Smriti Poudel

Spina Bifida
• Spinal Bifida, also called spinal dysraphia,
is a defective closure of the spinal cord or
vertebral column in which the posterior
portion of the laminae of the vertebrae fails
to close
• Lumbosacral area is the most common
site
 Types
Several types of the spina bifida are recognized, of which the
following three types are most common.

Spina bifida Occulta

• The defect is only in the vertebrae.
• The spinal cord and meninges are normal.
• Not visible externally

Meningocele
• The meninges protrude through the opening in the spinal canal.
• This forms a cyst filled with CSF and covered with skin.
• Visible defect, the protruded cyst encases meninges and spinal
fluid but no neural elements.
• It may be with or without neurological defects
• The sac may ruptures resulting subsequent infections.
Myelomeningocele (Or Meningomyeloceles):
• It is a protusion of cyst which encases
meninges, spinal fluid and nerves
• The meningomyeloceles are covered by a
thin membrane.
• Usually associated with neurological deficit
• The sac may leak in utero or may rupture
after birth, allowing free drainage of CSF.
This renders the child highly susceptible to
meningitis.
• Occurs four to five times more frequently
than meningocele
 Pathophysiology

• Arrest in the orderly formation of the

vertebral arches and spinal cord and
incomplete closure of the neural tube that
occurs between the fourth and sixth week
of embryogenesis.
 Etiology
1. Main cause is unknown
2. Nutritional deficiency especially folic acid
3. It is thought to result from genetic predisposition
triggered by the environment.
4. Certain drugs Including valporic acid, have
been known to cause neural tube defect if
administered during pregnancy.
5. Women who have spina bifida and parents who
have one affected child have an increased risk
of producing children with neural tube defects.
6. Radiation exposure
 Clinical Manifestations
Spina bifida occulta
• Most patient have no symptoms
– They have dimple in the skin or growth of hair over the
malformed vertebra i.e tuft of hair
– There is no externally visible sac

• With growth, the child may develop foot weakness or

bowel and bladder sphincters disturbances
• Progressive disturbances of gait
 Contd..
Meningocele
• An external cystic defect can be seen in the spinal
cord, usually midline.

• The sac is composed only of meninges and is filled

with CSF

• The cord and nerve root are usually normal

• There is seldom evidence of weakness of the legs or

lack of sphincter control.
• Sensory disturbances and parallel motor
dysfunction
• Sometimes associated with hip
dislocation, kyphosis, valgus or varus
 Contd

Myelomeningocele
• Hydrocephalus 2/3 of such cases

• Loss of motor control and sensation below the level of

lesion

• Contracture may occurs in knees ankles, or hips.

• Hip may be dislocated

• Club feet are associated commonly.

 Contd…
• Bladder dysfunction because sacral nerves that
innervate the bladder are affected.

• Fecal incontinence and constipation due to poor

innervations of the anal sphincter and bowel
musculature.

• Average intellectual ability . The developmental

disabilities includes
– Poor gross motor development
– Speech delays.
 Diagnostic Evaluation

• Prenatal detection is done through prenatal

ultrasound, maternal serum alpha fetoprotein
(MSAFP) screening which should be offered to all
women at risk.

• Diagnosis is primarily based on clinical

manifestations.

• CT scan and MRI may be performed to evaluate

further the brain and spinal cord.
 Management
• No treatment is required if spinal bifida
occulta is present without neurological
deficit
• Meningocele and meningomyelocele
requires multidisciplinary approach
• Management includes neurosurgical and
plastic surgical procedures to close the
defects without disturbing the neural
elements or removing any portion of the
nerves
• Procedure: Laminectomy and closure of the
open lesion or removal of sac can be done
soon after birth
• Also includes management of genitourinary
function like clean intermittent
catheterization, urinary diversion according
to condition and measures to control bowel
function
• Prophylaxis antibiotics are used to prevent
infection
 Prognosis
• Influenced by site of lesion and presence and degree
of associated hydrocephalus. Generally the higher the
defect, the greater the extent of neurological deficit and
greater likelihood of hydrocephalus.

• In absence of treatment most infants with

meningomyocele die early in infancy.

• Even with surgery, infants may manifest associated

neurosurgical, orthopedic, of urologic problems.

• New technique of treatment, research and improved

services has increased the life expectancy and has
greatly enhanced quality of life for children who receive
Rx.
 Complications
• Hydrocephalus

• Scoliosis, contractures and joint dislocation.

• Skin breakdown in sensory denervated areas

 Nursing Management
Assessment
Sensory motor response of lower extremities

Ability to void spontaneously, retention of urine,

symptoms of UTI.

Usual stooling pattern, need for medication to facilitate

elimination.

Mobility and use of braces, cast and other special

equipments.
 Nursing Diagnosis
Neonate (Preoperative)
• Risk for impaired skin integrity related to impaired motor and
sensory function

• Risk for infection related to contamination of the

myelomeningocele site

• Impaired urinary elimination related to neurological deficit

• Ineffective cerebral tissue perfusion related to potential

hydrocephalus

• Anxiety and fear of parents related to neonate with

neurological disorder and to surgery
 Nursing Diagnosis Contd…….

Infant and children (Postoperative)

• Ineffective thermoregulation following surgery

• Impaired urinary elimination related to sacral

denervation.

• Bowel incontinence of constipation relate to

impaired innervations of anal sphincter and bowel
musculature

• Disturbed body image related to the Childs

appearance, difficulties with locomotion, and lack of
control over excretory functions.
 Nursing interventions
In Neonates
Preventing skin integrity
• Avoid positioning on the infants back to
prevent pressure on the sac.

• Observe sac frequently for sign of irritation or

leakage of CSF

• Do not place diaper of covering directly over

the sac
 Contd…
• Use prone positioning with hips slightly flexed to
decrease tension on the sac.

• Provide meticulous skin care to all areas of the

body, especially ankles, knees, tip of the nose,
cheeks, and chin.

• Provide passive ROM exercises for muscle and joint

that infant does not use spontaneously, but avoid
hip exercise unless recommended

• Use foam pad to reduce pressure of mattress

against the skin
 Contd…
Preventing infection
• Be aware that infection of the sac is most commonly
caused by contamination by urine and feaces.
• Keep the buttock and genitalia clean
• Do not use diaper if the defect is in lower portion of
spine.
• Small piece of plastic taped between the defect and
anus helps to prevent contamination
• Apply sterile gauze or towel or moistened dressing
over the sac which should be changed frequently
• Monitor and report any signs of infections, oozing of
fluid or pus from the sac, fever, irritability or
listlessness, seizure.
Promoting Urinary elimination
Contd…
• Use Credes method to empty bladder and teach parents
the techniques. Apply firm gentle pressure to the
abdomen, beginning in the umbilical area and progressing
towards the symphasis pubis and continue the procedure
as long as the urine can be manually expressed

• Ensure fluid intake to dilute the urine

• Administer prescribed prophylactics antibiotics

• Monitor or report concentrated of foul smelling urine.

 Contd…
Maintaining cerebral tissue perfusion
• Monitor signs of hydrocephalus and report
and manage immediately
1. Irritability
2. Feeding difficulty, vomiting, decrease
appetite,
3. Temperature fluctuation
4. Decreased alertness
5. Tense fontanel
6. Increased head circumferences
 Contd…
Reducing fear
• Encourage parents to express feeling of
guilt, fear, lack of control, or helplessness.

• Provide accurate information about spina

bifida, and what to expect postoperatively

• Include the parents in all of infants care

and encourage private bonding time
 In Infants and children
Maintaining thermoregulation and preventing
complications

• Frequently monitor vital signs, skin color, level of

responsiveness

• Use an warmer to maintain temperature

• Prevent respiratory complications by changing

position frequently and preventing abdominal
distention

• Have o2 available.
 Contd….
• Maintain hydration and nutritional status with IV
or NG or oral feeding as prescribed.

• Keep surgical dressing clean, dry and observe

drainage

• Monitor and teach parents the signs of

hydrocephalus

• Prevent or limit direct contact with latex materials

to prevent allergy.
 Contd….
Achieving continence
• Teach parents or children about clean
intermittent self-catheterization

• Teach about the medications used to retain

urine rather than dribbling.

• Teach signs of UTI and proper management.

 Contd….
Achieving regular bowel elimination.

• Assist with bowel training program to compensate

for decreased sacral sensation

• Place the child in toileting schedule and taught to

push

• Use stool softener, enemas initially to fix schedule

• Prevent constipation and enhance fecal control.

 Contd….
Fostering positive body image
• Emphasis of strength and minimize disabilities

• Reassess functional ability continuously consult

frequently wit concerned person to increase
function

• Encourage participation with peers, relatives,

social gathering and Childs interest

• Rehabilitate the bowel and bladder by reassessing

periodically
 Contd…..
Community and homecare
• Identify home environment

• Teach the family how to clean and reuse

urinary catheter

• Instruct the parents to notify the signs of

associated problems to health care provider eg
UTI, hydrocephalus, infection, latex sensitivity
Thank you