SPINA BIFIDA

Introduction
Spina bifida is a birth defect that occurs when the spine and spinal cord don't form properly. It's a
type of neural tube defect. The neural tube is the structure in a developing embryo that
eventually becomes the baby's brain, 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 doesn't close or develop
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 necessary, early treatment for spina bifida involves surgery — although
such treatment doesn't always completely resolve the problem.

Types

Spina bifida (myelomeningocele)

Spina bifida can occur in different types: spina bifida
occulta, myelomeningocele (my-uh-lo-muh-NING-
go-seel) or the very rare type meningocele (muh-
NING-go-seel).

Spina bifida occulta

"Occulta" means hidden. It's the mildest and most

common type. 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.

Myelomeningocele

Also known as open spina bifida, myelomeningocele is the most severe type. 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 and may also cause paralysis
and bladder and bowel dysfunction.
Etiology
The exact cause of spina bifida remains a mystery.  No one knows what disrupts complete
closure of the neural tube, causing this malformation to develop.  Scientists suspect the factors
that cause spina bifida are multiple: genetic, nutritional, and environmental factors all play a
role.  Research studies indicate that insufficient intake of folic acid—a common B vitamin—in
the mother’s diet is a key factor in causing spina bifida and other neural tube defects.   Prenatal
vitamins typically contain folic acid as well as other vitamins. 
Epidemiology
The average worldwide incidence of spina bifida is 1 case per 1000 births, but marked
geographic variations occur. Neural tube defects occur at frequencies (per 10,000 births) ranging
from 0.9 in Canada and 0.7 in central France to 7.7 in the United Arab Emirates and 11.7 in
South America.
Health issues are different for each person with spina bifida. Open spina bifida
(myelomeningocele)—in which the spinal cord is exposed—tends to cause more severe
problems.1

Hydrocephalus
 Many infants born with spina bifida get extra fluid in and around the brain, a condition
called hydrocephalus, or water on the brain. The extra fluid can cause swelling of the
head, which may lead to brain injury.

Chiari II Malformation
 The brains of most children with open spina bifida are positioned abnormally. The lower
part of the brain rests farther down than normal, partially in the upper spinal canal. The
cerebrospinal (pronounced suh-ree-broh-SPAHYN-l) fluid can get blocked and cause
hydrocephalus. While most affected children have no other symptoms, a few may have
upper body weakness and trouble breathing and swallowing.

Tethered Spinal Cord

 Typically, the bottom of the spinal cord floats freely in the spinal canal, but for many
people with spina bifida, the spinal cord is attached to the spinal canal. Thus, the spinal
cord stretches as a person grows, and this stretching can cause spinal nerve damage. The
person might have back pain, scoliosis (crooked spine, pronounced skoh-lee-OH-sis),
weakness in the legs and feet, bladder or bowel control problems, and other issues.

Paralysis, Mobility Limitations

 People with spina bifida high on the back (near the head, for instance) might not be able
to move their legs. People with spina bifida low on the back (near the hips, for example)
 might have some leg mobility and be able to walk unassisted or with crutches, braces, or
walkers.

Lack of Bladder and Bowel Control

 People with spina bifida often cannot control their bladder and bowel movements. They
also can develop urinary tract infections.

Latex Allergy
 Many people with spina bifida—possibly three-quarters of those with the condition—are
allergic to latex, or natural rubber. While researchers still don’t entirely understand why
the rate in those with spina bifida is so high, some experts believe such an allergy can be
caused by frequent exposure to latex, which is common for people with spina bifida who
have shunts and have had many surgeries.2

Learning Disabilities
 While at least 80% of children with open spina bifida do not have learning deficiencies,
some do have learning problems.3

Other Conditions
 Some people with open spina bifida have additional physical and psychological
conditions, including digestive, vision, sexual, social, and emotional problems; obesity;
and depression.

Pathophysiology
 Spina bifida occurs when local regions of the neural tube fail to fuse or there is failure in
formation of the vertebral neural arches. Neural arch formation occurs in the first month
of embryonic development (often before the mother knows she is pregnant). Some forms
are known to occur with primary conditions that cause raised central nervous system
pressure, raising the possibility of a dual pathogenesis.
 In normal circumstances, the closure of the neural tube occurs around the 23rd (rostral
closure) and 27th (caudal closure) day after fertilization. However, if something
interferes and the tube fails to close properly, a neural tube defect will occur. Medications
such as some anticonvulsants, diabetes, obesity, and having a relative with spina bifida
can all affect the probability of neural tube malformation.
 Extensive evidence from mouse strains with spina bifida indicates that there is sometimes
a genetic basis for the condition. Human spina bifida, like other human diseases, such
as cancer, hypertension and atherosclerosis (coronary artery disease), likely results from
the interaction of multiple genes and environmental factors.
 Research has shown the lack of folic acid (folate) is a contributing factor in the
pathogenesis of neural tube defects, including spina bifida. Supplementation of the
mother's diet with folate can reduce the incidence of neural tube defects by about 70%
 and can also decrease the severity of these defects when they occur. It is unknown how or
why folic acid has this effect.
 Spina bifida does not follow direct patterns of heredity as do muscular
dystrophy or hemophilia. Studies show a woman having had one child with a neural tube
defect such as spina bifida has about a 3% risk of having another affected child. This risk
can be reduced with folic acid supplementation before pregnancy. For the general
population, low-dose folic acid supplements are advised (0.4 mg/day)

Clinical manifestations
 Asymptomatic
 Neurological deficits are present below the level of the lesion
 Lower limb paralysis
 Musculoskeletal deformities
 Incompetence of anal and bladder sphincters
 CSF leakage may present and there are chances of infections

Signs and symptoms

A newborn with this disorder will have an open area or a fluid-filled sac on the mid to lower
back.

Symptoms may include:

o Loss of bladder or bowel control

o Partial or complete lack of sensation
o Partial or complete paralysis of the legs
o Weakness of the hips, legs, or feet of a newborn

Other signs and/or symptoms may include:

o Abnormal feet or legs, such as clubfoot

o Buildup of fluid inside the skull (hydrocephalus)

 Spina bifida occulta. Typically, there aren't any signs or symptoms because the spinal
nerves aren't involved. But you can sometimes see signs on the newborn's skin above the
spinal defect, including an abnormal tuft of hair, or a small dimple or birthmark.
Sometimes, the skin marks can be signs of an underlying spinal cord issue that can be
discovered with MRI or spinal ultrasound in a newborn.
 Myelomeningocele. In this severe type of spina bifida:
o The spinal canal remains open along several vertebrae in the lower or middle back
o Both the membranes and the spinal cord or nerves protrude at birth, forming a sac
 o Tissues and nerves usually are exposed, though sometimes skin covers the sac

Diagnostic evaluation
Approximately 90 per cent of cases of spina bifida are detected with an ultrasound scan before
18 weeks of pregnancy. Other tests used to diagnose spina bifida are maternal blood tests which
measure alpha-fetoprotein (AFP), and magnetic resonance imaging (MRI) scans.

If spina bifida is present, specialist obstetric care and support will be provided. Consultation with
an expert pediatrician is available at both the Royal Children’s Hospital and Monash Children’s
Hospital.

In open spina bifida where the cord and nerves are exposed (called spina bifida aperta), it is
important to close the defect within the first few days of life to avoid infection, excess drainage
of cerebrospinal fluid and further damage to the spinal cord and nerves.

Occasionally, spina bifida is not detected until birth when a large soft lump or skin covered
lesion on the baby’s back is noticed. This lump contains spinal cord, nerves and often fatty tissue
(called a lipomeningocoele). The need for surgery in this situation is not urgent, because the
spinal cord and nerves are not exposed.

Medication
The medications used most frequently in myelomeningocele are for treatment of neurogenic
bladder dysfunction. These medications are used in conjunction with some form of bladder
emptying technique to prevent upper urinary tract complications and to facilitate social
continence. Among the drugs used are the following:
o Anticholinergics (oxybutynin chloride, hyoscyamine sulfate)
o Tricyclic Antidepressants
o Alpha-Adrenergic Antagonists.
o Antispasmodic Agents

Treatment
Spina bifida treatment depends on the severity of the condition. Spina bifida occulta often doesn't
require any treatment at all, but other types of spina bifida do.

Surgery before birth

Nerve function in babies with spina bifida can worsen after birth if spina bifida isn't treated.
Prenatal surgery for spina bifida (fetal surgery) takes place before the 26th week of pregnancy.
Surgeons expose the pregnant mother's uterus surgically, open the uterus and repair the baby's
spinal cord. In select patients, this procedure can also be performed less invasively with a
fetoscope through ports in the uterus.

Research suggests that children with spina bifida who had fetal surgery may have reduced
disability and be less likely to need crutches or other walking devices. Fetal surgery may also
reduce the risk of hydrocephalus. Ask your doctor whether this procedure may be appropriate for
you. Discuss the potential benefits and risks, such as possible premature delivery and other
complications, for you and your baby.

It's important to have a comprehensive evaluation to determine whether fetal surgery is feasible.
This specialized surgery should only be done at a health care facility that has experienced fetal
surgery experts, a multispecialty team approach and neonatal intensive care. Typically, the team
includes a fetal surgeon, pediatric neurosurgeon, maternal-fetal medicine specialist, fetal
cardiologist and neonatologist.

Cesarean birth

Many babies with myelomeningocele tend to be in a feet-first (breech) position. If your baby is
in this position or if your doctor has detected a large cyst or sac, cesarean birth may be a safer
way to deliver your baby.

Surgery after birth

Myelomeningocele requires surgery. Performing the surgery early can help minimize the risk of
infection associated with the exposed nerves. It may also help protect the spinal cord from more
trauma.

During the procedure, a neurosurgeon places the spinal cord and exposed tissue inside the baby's
body and covers them with muscle and skin. At the same time, the neurosurgeon may place a
shunt in the baby's brain to control hydrocephalus.

Treatment for complications

In babies with myelomeningocele, irreparable nerve damage has likely already occurred and
ongoing care from a multispecialty team of surgeons, physicians and therapists is usually needed.
Babies with myelomeningocele may need more surgery for a variety of complications. Treatment
for complications — such as weak legs, bladder and bowel problems, or hydrocephalus —
typically begins soon after birth.

Depending on the severity of spina bifida and the complications, treatment options may include:

 Walking and mobility aids. Some babies may start exercises to prepare their legs for
walking with braces or crutches when they're older. Some children may need walkers or a
 wheelchair. Mobility aids, along with regular physical therapy, can help a child become
independent. Even children who need a wheelchair can learn to function very well and
become self-sufficient.
 Bowel and bladder management. Routine bowel and bladder evaluations and
management plans help reduce the risk of organ damage and illness. Evaluations include
X-rays, kidney scans, ultrasounds, blood tests and bladder function studies. These
evaluations will be more frequent in the first few years of life but less often as children
grow. A specialist in pediatric urology with experience in evaluating and performing
surgery on children with spina bifida may offer the most effective management options.
o Bowel management may include oral medications, suppositories, enemas, surgery
or a combination of these approaches.
o Bladder management may include medications, using catheters to empty the
bladder, surgery or a combination of treatments.
 Surgery for hydrocephalus. Most babies with myelomeningocele will need a surgically
placed tube that allows fluid in the brain to drain into the abdomen (ventricular shunt). This
tube might be placed just after birth, during the surgery to close the sac on the lower back
or later as fluid accumulates. A less invasive procedure, called endoscopic third
ventriculostomy, may be an option. But candidates must be carefully chosen and meet
certain criteria. During the procedure, the surgeon uses a small video camera to see inside
the brain and makes a hole in the bottom of or between the ventricles so cerebrospinal fluid
can flow out of the brain.
 Treatment and management of other complications. Special equipment such as bath
chairs, commode chairs and standing frames may help with daily functioning. Whatever the
issue — orthopedic complications, tethered spinal cord, GI issues, skin problems or others
— most spina bifida complications can be treated or at least managed to improve quality of
life.
Ongoing care

Children with spina bifida need close follow-up care and observation. Their primary care doctors
evaluate growth, the need for vaccinations and general medical issues, and they coordinate
medical care.

Children with spina bifida also often need treatment and ongoing care from:

 Physical medicine and rehabilitation

 Neurology
 Neurosurgery
 Urology
  Orthopedics
 Physical therapy
 Occupational therapy
 Special education teachers
 Social workers
 Dietitians

Complication
 Traumatic birth and difficult delivery of the baby
 Frequent urinary tract infections
 Fluid buildup on the brain (hydrocephalus)
 Loss of bowel or bladder control
 Brain infection (meningitis)
 Permanent weakness or paralysis of legs
 This list may not be all-inclusive.

Nursing intervention
Nursing care planning goals for clients with spina bifida include prevent infection, maintain skin
integrity, prevent trauma related to disuse, increase family coping skills, education about the
condition, and support.
Here are seven (7) nursing care plans (NCP) and nursing diagnosis (NDx) for spina bifida:
 Hypothermia
 Impaired Urinary Elimination
 Bowel Incontinence
 Disturbed Body Image
 Interrupted Family Processes
 Risk for Infection
 Risk for Injury

Discharge planning
Medication
Instructed to:
 Take the medications religiously
 Take the antibiotics with meals
  Take the medications on time without any lapses
 Educate the significant others about drugs as well its effect, indications, adverse effects
and what to do when it occurs.
 Take home meds:
1. Cefixime 100mg/5ml 7ml BID
2. Paracetamol 250mg/ 5ml 5ml q 4 PRN for fever
Exercise
 Encourage to resume normal daily activities
 Encourage to exercise lower extremities by walking
 Encourage ambulation for faster recovery of damaged tissues
 Encourage passive range of motion exercise to strengthen muscles
Treatment
 Encourage to follow the treatment regimen prescribed by the doctor
 Explain the significant others the importance of drug compliance
 Explain to the significant others, in their level of understanding about the condition of the
patient.
Hygiene
Encourage to:
 Have daily hygiene
 Clean the surgical site and always keep it dry and clean
 Wash hands before and after eating and/or in contact with dirty objects
Outpatient visit
 Instruct to return for follow up check-up the OPD
 Instruct to monitor or watch closely for any unusual ties such as infections, bowel
problems, cough and colds and report to their physician
Diet
 Instructed to continue to the usual diet
 Encouraged to eat foods rich in protein to aid in the healing of the wounds
 Encouraged to eat nutritious foods such as fruits and vegetables for faster growth and
development of the child.
Prognosis
 A myelomeningocele can most often be surgically corrected, but the affected nerves may
still not function normally. The higher the location of the defect on the baby's back, the
more nerves will be affected.
 With early treatment, length of life is not severely affected. Kidney problems due to poor
drainage of urine are the most common cause of death.
  Most children with myelomeningocele will have normal intelligence. However, because
of the risk of hydrocephalus and meningitis, more of these children will have learning
problems and seizure disorders.
 New problems within the spinal cord can develop later in life, especially after the child
begins growing rapidly during puberty. This can lead to more loss of function as well as
orthopedic problems such as scoliosis, foot or ankle deformities, dislocated hips, and
joint tightness or contractures.
 Many people with myelomeningocele primarily use a wheelchair.

https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/spina-bifida

https://www.medicalnewstoday.com/articles/220424.php#symptoms

https://en.wikipedia.org/wiki/Spina_bifida#Pathophysiology

https://www.mayoclinic.org/search/search-results?q=spina%20bifida

https://nurseslabs.com/7-spina-bifida-nursing-care-plans/

https://www.nichd.nih.gov/health/topics/spinabifida/conditioninfo/disorders-conditions