2. DEFINITION
Congenital disorders can be defined as structural or functional
anomalies that occur during intrauterine life.
Also called birth defects, congenital anomalies or congenital
malformations, these conditions develop prenatally and may be
identified before or at birth, or later in life.
3. INCIDENCE
An estimated 6% of babies worldwide are born with a congenital
disorder, resulting in hundreds of thousands of associated deaths.
However, the true number of cases may be much higher because
statistics do not often consider terminated pregnancies and
stillbirths.
4. CNS CONGENITAL ANOMALIES
CNS congenital anomalies are birth defects of the physical structure
of the brain or spinal cord that develop in utero, or when a fetus is
developing during pregnancy.
6. Introduction
Spina bifida is a condition that the backbone that protects the
spinal cord does not form and close properly.
This often results in damage to the spinal cord and nerves.
Spina bifida might cause physical and intellectual disabilities that
range from mild to severe.
7. Definition
Spina bifida is a birth defect in which there is incomplete closing of
the spine and the membranes around the spinal cord during early
development in pregnancy.
Spina bifida is a condition that affects the spine and is usually apparent at
birth.
Incidence is 1-5 per 1000 live births
8. Causes
The exact cause is unknown
Risk factors are
Consanguinity marriage
Drugs (E.g. valproate)- anticonvulsant (or anti-epileptic) medicine.
Folic acid deficiency
Exposure to chemicals and radiation during antenatal period.
9. CLASSIFICATION OF SPINA BIFIDA
1. Spina Bifida Occulta
2. Spina Bifida Cystica
a. Meningocoele
b. Myelomeningocele
10. CLASSIFICATION OF SPINA BIFIDA
SPINA BIFIDA OCCULTA
Spina bifida occulta is the mildest type of spina bifida.
The word “occulta” means “hidden.”
Spina bifida occulta is also known as “hidden spina bifida”.
With it, there is a small gap in the spine, but no opening or sac on the back.
The spinal cord and the nerves usually are normal.
The defects is not visible externally
11. SPINA BIFIDA OCCULTA
Sometimes, spina bifida occulta is
indicated by a visible sign on the
skin directly over the defect.
Examples include a patch of thick
hair, a growth, an unusual pigment,
an extremely large dimple, or a pad
of fat.
12. CLASSIFICATION OF SPINA BIFIDA
SPINA BIFIDA CYSTICA
Spina bifida cystica classified into two
a . Meningocele
b. Myelomeningocele
13. CLASSIFICATION OF SPINA BIFIDA
A.MENINGOCELE
It is a condition in which the meninges herniates/protrudes through the
gap in the spine.
This creates a sac filled with fluid (called a Meningocele) on the baby’s
back.
There’s usually little or no nerve damage
The sac containing meninges and CS.
15. CLASSIFICATION OF SPINA BIFIDA
B. MYELOMENINGOCELE
Myelomeningocele is the most serious type.
It also is known as open spina bifida.
It is a condition that the meninges herniates through the gap
in the spine with nerves.
There’s usually nerve damage
The sac contains meninges, CSF and spinal nerves
17. CLINICAL MANIFESTATIONS
Spina bifida occulta:
Children diagnosed with spina bifida occulta might have a visible
mark on their skin at the lower part of the back when they’re born
that appears as a lump, a red or purple patch (hemangioma) or a dark
spot (birthmark)
19. CLINICAL MANIFESTATIONS
Spina Bifida Cystica
Sensory disturbances
Weakness
Partial paralysis of lower extremities
Overflow incontinence with constant dribbling of urine
Hydrocephalus
Lack of bowel control
20. Diagnosis
Maternal serum alpha-fetoprotein (MSAFP) test.
A sample of the mother's blood is drawn and tested for alpha-
fetoprotein(a protein that develops in the fetus's liver)
An abnormally high levels of AFP suggest that the baby has a neural
tube defect(birth defects of the brain, spine or spinal cord)
(normal AFP level - 10 to 150 ng/mL)
21. Diagnosis
Ultrasound
Fetal ultrasound is the most accurate
method to diagnose spina bifida.
Investigations such as X-ray, MRI, or
CT, to get a clearer view of the baby’s
spine and the bones in the back.
22. MANAGEMENT
Surgical correction
The spinal cord and its nerve roots are put back inside the spine and
covered with meninges.
Management is complicated and should involve a multidisciplinary
team.
Team should include pediatrician, orthopaedic surgeon, neurologist,
physiotherapist etc.
24. PREVENTION
Folic acid supplementation
Dietary supplementation with folic acid has been shown to be
helpful in preventing spina bifida.
Sources of folic acid include whole grains, fortified breakfast cereals,
dried beans, leafy vegetables and fruits.
25. NURSING MANAGEMENT
PREOPERATIVE CARE
Kept flat on his abdomen with a single
layer of sterile gauze.
The genitalia and buttocks must be kept
clean.
The ankles to be supported with foam
rubber pads
Antibiotics must be given as order if
infection is suspected.
26. NURSING MANAGEMENT
Emptying the infant’s bladder every 2 hours during the day and once
at night.
If evidence of urinary infection occurs culture should be done to
determine the antibiotics.
The infant to be feeding properly .
All the vital signs should be taken and recorded.
27. NURSING MANAGEMENT
POSTOPERATIVE CARE
The nurse is responsible for observing
Temperature, Pulse, Respiration, symptoms of
shock etc.
Head circumference of the infant must be
measured frequently.
Surgical dressing should be kept clean.
Fluid and electrolyte management
28. NURSING DIAGNOSES
Impaired physical mobility related to neuromuscular impairment
Bowel incontinence related to neuromuscular impairment
Impaired urinary elimination related to neuromuscular impairment
Body image disturbances related to biophysical factors of child
Altered family process related to situational crisis of long term condition of child
Impaired skin integrity related to skeletal prominence
