SPINA BIFIDA

Definition: Spina bifida, also called spinal dysraphia, is a malformation of the spine in
which the posterior portion of the laminae of the vertebrae fails to close. It occurs in
approximately 1 per 1,000 live births in the United States and is the most common
developmental defect of the central nervous system (CNS). It is more common in
Caucasian than in nonCaucasian people.

(A) Normal spine. (B) Spina bifida occulta. (C) Spina bifida with meningocele. (D)
Spina bifida with myelomeningocele.

Spina Bifida Occulta

The defect is only in the vertebrae. The spinal cord and meninges are normal.

Meningocele
The meninges protrude through the opening in the spinal canal. This forms a cyst filled
with CSF and covered with skin.

Myelomeningocele (or Meningomyelocele)

The spinal cord and cord membranes protrude through the defect in the laminae of the
vertebral column. Myelomeningoceles are covered by a thin membrane.
 PATHOPHYSIOLOGY AND ETIOLOGY

1. Unknown etiology but generally thought to result from genetic predisposition triggered
by something in the environment
A. Certain drugs, including valproic acid, have been known to cause neural tube
defects if administered during pregnancy.
B. Women who have spina bifida and parents who have one affected child have an
increased risk of producing children with neural tube defects.
2. Involves an arrest in the orderly formation of the vertebral arches and spinal cord that
occurs between the fourth and sixth weeks of embryogenesis.
3. Theories of causation include:
A. There is incomplete closure of the neural tube during the fourth week of
embryonic life.
B. The neural tube forms adequately, then ruptures.
4. In spina bifida occulta, the bony defect may range from a very thin slit separating one
lamina from the spinous process to a complete absence of the spine and laminae.
A. A thin, fibrous membrane sometimes covers the defect.
B. The spinal cord and its meninges may be connected with a fistulous tract
extending to and opening onto the surface of the skin.
5. In meningocele, the defect may occur anywhere on the cord. Higher defects (from
thorax and upward) are usually meningoceles.
A. Surgical correction is necessary to prevent rupture of the sac and subsequent
infection.
B. Prognosis is good with surgical correction.
6. In myelomeningocele (meningomyelocele), the lesion contains both the spinal cord and
cord membranes.
A. A bluish area may be evident on the top because of exposed neural tissue.
B. The sac may leak in utero or may rupture after birth, allowing free drainage of
CSF. This renders the child highly susceptible to meningitis.
C. Occurs four to five times more frequently than meningocele.

SPINA BIFIDA

In utero fetal development

Failure of spinous process to Defect in closure of neural tube

join lumbosacral area
 Complete nonclosure of neural
Spina bifida occulta Protruding sac through tube with protruding sac
defect-containing meninges through defect containing parts
of spinal cord

Skin depression or simple

port wine angioma tufts of
hair subcutaneous lipomas
Myelomeningocele
Meningocele

Bowel and bladder sphincter

weakness (persistent or intermittent
enuresis) foot weakness, gait
disturbance (late walking)

Surgical repaid and V-P shunt

based on type and extent of defect
and deficit

Immobility or mobility with

Resolution of defect and minor braces and crutches
deficits

Drug Alert
Maternal periconceptional use of folic acid supplementation reduces by 50% or more the
incidence of neural tube defects in pregnancies at risk.

CLINICAL MANIFESTATIONS
Spina Bifida Occulta

1. Most patients have no symptoms.

A. They may have a dimple in the skin or a growth of hair over the
malformed vertebra.
B. There is no externally visible sac.
2. With growth, the child may develop foot weakness or bowel and bladder sphincter
disturbances.
3. This condition is occasionally associated with more significant developmental
abnormalities of the spinal cord, including syringomyelia and tethered cord.

Meningocele

1. An external cystic defect can be seen in the spinal cord, usually in the midline.
A. The sac is composed only of meninges and is filled with CSF.
B. The cord and nerve roots are usually normal.
2. There is seldom evidence of weakness of the legs or lack of sphincter control.

Myelomeningocele

1. A round, raised, and poorly epithelialized area may be noted at any level of the spinal
column. However, the highest incidence of the lesion occurs in the lumbosacral area.
2. Hydrocephalus occurs in approximately two-thirds of children with myelomeningocele
due to associated Arnold-Chiari malformation, which causes a block in the flow of
CSF through the ventricles.
3. Loss of motor control and sensation below the level of the lesion can occur. These
conditions are highly variable and depend on the size of the lesion and its position on
the cord.

A. A low thoracic lesion may cause total flaccid paralysis below the waist.
B. A small sacral lesion may cause only patchy spots of decreased sensation in the
feet.

4. Contractures may occur in the ankles, knees, or hips. Hips may become dislocated.
A. Nature and degree of involvement depend on size and location of lesion.
B. This occurs because some fibers of innervation do get through. One side
of a hip, knee, or ankle may be innervated while the opposing side may
not be. The unopposed side then becomes pulled out of position.
5. Clubfeet are a common accompanying anomaly; thought to be related to the position of
paraplegic feet in the uterus.
6. Bladder dysfunction because sacral nerves that innervate the bladder are affected. The
bladder fails to respond to normal messages that it is time to void and simply fills and
overflows, causing incontinence and susceptibility to urinary tract infections (UTIs)
because of incomplete emptying.
7. Fecal incontinence and constipation are caused by poor innervation of the anal
sphincter and bowel musculature.
8. Most children have average intellectual ability despite hydrocephalus. Developmental
disabilities include the following:

A. Gross motor development children will need assistance in gaining and

maintaining mobility.
B. Speech delays due to frequent hospitalizations and increased intracranial pressure
due to hydrocephalus.
C. Most children are able to learn in a mainstream school environment, provided
they are able to overcome other barriers (architectural and attitudinal).

Diagnostic Evaluation

1. Prenatal detection is done through prenatal ultrasound and fetal MRI. This testing
should be offered to all women at risk (women who are affected or have had other
affected children).
2. Diagnosis is primarily based on clinical manifestations.
3. CT scan and MRI may be performed to evaluate further the brain and spinal cord.

MANAGEMENT
Surgical Intervention

1. Procedure: laminectomy and closure of the open lesion or removal of the sac usually
can be done soon after birth.
2. Purpose:
A. To prevent further deterioration of neural function.
B. To minimize the danger of rupture and infection, especially meningitis.
C. To improve cosmetic effect.
D. To facilitate handling of the infant.

Multidisciplinary Follow-Up for Associated Problems

1. A coordinated team approach will help maximize the physical and intellectual
potential of each affected child.
2. The team may include a neurologist, neurosurgeon, orthopedic surgeon, urologist,
primary care provider, social worker, physical therapist, a variety of community-
based and hospital staff nurses, and the child and family.
3. Numerous neurosurgical, orthopedic, and urologic procedures may be necessary to
help the child achieve maximum potential.

Prognosis
1. Influenced by the site of the lesion and the presence and degree of associated
hydrocephalus. Generally, the higher the defect, the greater the extent of neurologic
deficit and the greater the likelihood of hydrocephalus.
2. In the absence of treatment, most infants with meningo myelocele die early in infancy.
3. Surgical intervention is most effective if it is done early in the neonatal period,
preferably within the first few days of life.
4. Even with surgical intervention, infants can be expected to manifest associated
neurosurgical, orthopedic, or urologic problems.
5. New techniques of treatment, intensive research, and improved services have increased
life expectancy and have greatly enhanced the quality of life for most children who
receive treatment.

Complications

1. Hydrocephalus associated with meningocele; may be aggravated by surgical repair.

2. Scoliosis, contractures, and joint dislocation.
3. Skin breakdown in sensory denervated areas and under braces.

Nursing Assessment

1. Assess sensory and motor response of lower extremities.

2. Assess ability to void spontaneously, retention of urine, symptoms of UTI.
3. Assess usual stooling patterns, need for medications to facilitate elimination.
4. Assess mobility and use of braces, casts, and other special equipment.

Nursing Diagnoses
Neonates (Preoperative)

1. Risk for Impaired Skin Integrity related to impaired motor and sensory function
2. Risk for Infection related to contamination of the myelomeningocele site
3. Impaired Urinary Elimination related to neurologic deficits
4. Ineffective Tissue Perfusion: Cerebral related to potential hydrocephalus
5. Fear (parents) related to neonate with neurologic disorder and to surgery

Infants and Children (Postoperative)

1. Ineffective Thermoregulation following surgery

2. Impaired Urinary Elimination related to sacral denervation
3. Bowel Incontinence or Constipation related to impaired innervation of anal sphincter
and bowel musculature
4. Disturbed Body Image related to the child's appearance, difficulties with locomotion,
and lack of control over excretory functions

NURSING INTERVENTIONS

Neonates
Protecting Skin Integrity

1. Avoid positioning on the infant's back to prevent pressure on the sac. Check position at
least once every hour.

2. Do not place a diaper or other covering directly over the sac.

3. Observe the sac frequently for evidence of irritation or leakage of CSF.

4. Use prone positioning with hips only slightly flexed to decrease tension on the sac.

5. Place a foam rubber pad covered with a soft cloth between the infant's legs to maintain
the hips in abduction and to prevent or counteract subluxation. A diaper roll or small
pillow may be used in place of the foam rubber pad.

6. Allow the infant's feet to hang freely over the pads or mattress edge to prevent
aggravation of foot deformities.

7. Provide meticulous skin care to all areas of the body, especially ankles, knees, tip of
nose, cheeks, and chin.

8. Provide passive ROM exercises for muscles and joints that the infant does not use
spontaneously. Avoid hip exercises because of common hip dislocation, unless
otherwise recommended.

9. Use foam or fleece pad to reduce pressure of the mattress against the infant's skin.

10. Avoid pressure on the infant's back during feeding by holding the infant with your
elbow rotated to avoid touching the sac, or feeding while infant is lying on side or
prone on your lap. Encourage the parents to use these positions to provide infant
stimulation and bonding.

Preventing Infection

1. Be aware that infection of the sac is most commonly caused by contamination by urine
and feces.
2. Keep the infant's buttocks and genitalia scrupulously clean.

A. Do not diaper the infant if the defect is in the lower portion of the spine.
B. Use a small plastic drape taped between the defect and the anus to help to prevent
contamination.
3. Apply a sterile gauze pad or towel or a sterile, moistened dressing over the sac as
directed.
A. When the sterile covering is used, it should be changed frequently to keep the area
free from exudate and to maintain sterility.
B. Care must be taken to prevent the covering from adhering to and damaging the
sac.
4. Monitor and report immediately any signs of infection.
A. Oozing of fluid or pus from the sac.
B. Fever.
C. Irritability or listlessness.
D. Seizure.

Promoting Urinary Elimination

1. Use Cred's method for emptying the bladder (unless contraindicated by vesicoureteral
reflux), and teach parents the technique.
2. Apply firm, gentle pressure to the abdomen, beginning in the umbilical area and
progressing toward the symphysis pubis.
3. Continue the procedure as long as urine can be manually expressed.
4. Ensure fluid intake to dilute the urine.
5. Administer prescribed prophylactic antibiotics.
6. Monitor and report concentrated or foul-smelling urine.

Maintaining Cerebral Tissue Perfusion

1. Monitor for signs of hydrocephalus, and report immediately.

A. Irritability.
B. Feeding difficulty, vomiting, decreased appetite.
C. Temperature fluctuation.
D. Decreased alertness.
E. Tense fontanelle.
F. Increased head circumference.

Reducing Fear

1. Encourage parents to express feelings of guilt, fear, lack of control, or helplessness.

2. Provide accurate information about spina bifida and what to expect postoperatively.
3. Include the parents in all of infant's care, and encourage private bonding time.

Infants and Children

Maintaining Thermoregulation and Preventing Complications
1. Frequently monitor temperature, pulse, respirations, color, and level of responsiveness
postoperatively, based on the infant's stability.
2. Use an Isolette or infant warmer to prevent temperature fluctuation.
3. Prevent respiratory complications.
A. Periodically reposition the infant to promote lung expansion.
B. Watch for abdominal distention, which could interfere with breathing.
C. Have oxygen available.
4. Maintain hydration and nutritional intake.

A. Administer I.V. fluids as ordered; keep accurate intake and output log.
B. Administer gavage feedings as ordered.
C. Begin bottle-feeding when infant is responsive and tolerating feedings. Give
small, frequent feedings slowly so air can be expelled naturally without bubbling.

5. Keep the surgical dressing clean and dry, and observe for drainage. Avoid pressure to
the area and diapers that cover the incision until healed.
6. Monitor for and teach parents to recognize signs of hydrocephalus. Report
immediately.
7. Limit or prevent direct contact of the child to products routinely used that contain latex,
due to risk of latex allergy and severe reaction. Latex products include BP cuffs,
tourniquets, tape, indwelling catheters, gloves, and I.V. tubing injection ports. Develop
protocols specifying modification of care for children at risk for latex allergy.

Nursing Alert
Be aware that children with spina bifida have a far greater risk for latex allergy than the
general population, estimated at up to 20% of spina bifida patients. Symptoms include
hives, itching, wheezing, and anaphylaxis. Incidence increases with time and may be
related to repeated exposure to products containing latex.

Achieving Continence

1. Teach parents that continence can usually be achieved with clean, intermittent self-
catheterization.
A. Children can generally be taught to catheterize themselves by age 6 or 7.
B. Parents can catheterize younger children.
2. Teach the following procedure:

A. Gather equipment: catheter, water-soluble lubricant, soap and water, urine

collection container.
B. Wash hands.
C. Position patient.
D. Clean the area around the urethral meatus.
E. Lubricate the catheter tip.
F. Insert the catheter until urine starts to flow. Have urine collection container or
diaper available.
G. Remove catheter when urine is drained from bladder.
H. Clean any lubricant off the child.
I. Dispose of urine.
J. Wash hands.

3. Teach about the action of medications, such as imipramine (Tofranil) and ephedrine
(Ephed II), if prescribed, which are used to help children retain urine rather than
dribbling. When used with self-catheterization, many children can stay dry for 3 to 4
hours at a time.
4. Teach the signs of UTI (concentrated, foul-smelling urine; irritability; pain or burning;
and fever) and the proper administration of antibiotics either prophylactically or when
prescribed for infection.
5. For children who cannot achieve urinary continence through intermittent
catheterization, provide information about options, such as surgically implanted
mechanical urinary sphincters and bladder pacemakers, indwelling catheters, external
collecting devices, and urinary diversion (may be necessary in some cases).

Achieving Regular Bowel Elimination

1. Assist with bowel training program to compensate for decreased sacral sensation.
A. Children are placed on a toileting schedule and are taught to push.
B. Medications, such as stool softeners, suppositories, or enemas, may be
used initially to help determine scheduling.
2. To prevent constipation and enhance fecal control, encourage intake of high-fiber,
high-fluid diet. Such medications as psyllium (Metamucil) may be used to increase
bulk or soften stool.

Fostering Positive Body Image

1. Emphasize rehabilitation that makes use of the child's strengths and minimizes
disabilities.
2. Continually reassess functional abilities, and offer suggestions to increase
independence. Periodically consult with physical or occupational therapists to help
maximize function.
3. Encourage the use of braces and specialized equipment to enhance ambulation while
minimizing the appearance of the equipment. For example, wear pants instead of
dresses or shorts to cover leg braces; choose a compact wheelchair that can be
decorated or personalized for the child.
4. Encourage participation with peer group and in activities that build on strengths, such
as cognitive abilities, interest in music, or art.
5. Periodically, reassess bowel and bladder programs. The ability to stay dry for
reasonable time intervals is one of the greatest factors in enhancing self-esteem and
positive body image.

Family Education and Health Maintenance

1. Prepare the parents to feed, hold, and stimulate their infant as naturally as possible.
2. Teach the parents the special techniques that may be required for holding and
positioning, feeding, caring for the incision, emptying the bladder, and exercising
muscles.
3. Alert the parents to safety needs of the child with decreased sensation, such as
protection from prolonged pressure, the risk of burns due to bath water that is too
warm, and avoidance of trauma from contact with sharp objects.
4. Urge continued follow-up and health maintenance, including immunizations and
evaluation of growth and development.
5. Advise parents that children with paralysis are at risk for becoming overweight due to
inactivity, so they should provide a low-fat, balanced diet; control snacking; and
encourage as much activity as possible.