Spina Bifida

Definition: - refers to malformations of the spine in which the posterior portion of the vertebral laminae fails to close. These malformations are thought to result from incomplete of defective closure of the neural tube during the fourth to sixth weeks of embryonic life. - most common site for spina bifida is the lower thoracic, lumbar or sacral region. Cause: - the etiology is unknown - genetic and environmental factors such as nutrition and exposure to harmful substances - multifactorial inheritance Risk Factors: - Previous babya and family history of spina bifida - maternal obesity - diabetes during pregnancy - maternal stress - women who has epileptic seizures - maternal fever in the first trimester

Pathophysiology: In utero fetal development

Failure of spinous process to join lumbosacral area

Defect in closure of neural tube

Spina bifida occulta

Protruding sac through defect-containing meninges

Complete nonclosure of neural tube with protruding sac through defect containing parts of spinal cord

Skin depression or simple port wine angioma tufts of hair subcutaneous lipoma

Meningocele Myelomeningocele

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

Resolution of defect and minor deficits

Immobility or mobility with braces and crutches

Three Major Types of Spina Bifida: y Spina bifida occulta - also known as hidden spina bifida - the most common type, the defects involves only the vertebrae - the spinal cord and meninges are normal and there is no opening on the back - there is no motor or sensory impairments at birth but neurologic deterioration is evident later in childhoold or adulthood - considered as a mild disease since there is no disturbance of spinal function and neurologic problems are rare - can be diagnosed at any age Meningocele - the meninges protrude the opening in the spinal cord, forming a cyst filled with cerebrospinal fluid (CSF) and covered with skin -defect may occur anywhere in the spinal cord. Higher defects (from thorax and upward) are usually meningoceles -Neurologic problems are rare, and no nerve damage Myelomeningocele - both the spinal cord and meninges protrude through the defect in the veretebral column and are protected by a thin, membranous sac - various forms of permanent neurologic deficit are present - causes nerve damage, and more sever disabilities - most have significant hydrocephalus - the sac may leak in utero or may rupture after birth, allowing free drainage of CSF. This renders the child highly susceptible to meningitis. -Myelomeningocele occurs 4 to 5 times more frequently than meningocele. In the absence of treatment, most infants with myelomeningocele die soon after birth. Encephalocele - also known as cranium bifidum - research has indicated that teratogens (substances known to cause birth defect), trypan blue (a stain used to color dead tissues or cells blue), and arsenic may damage the developing fetus and cause encephaloceles. - has sac like protrusions of the brain and the membranes that cover it through the openings in the skull - this type of spina bifida rarely occurs

Spina bifida is the most common developmental defect of the central nervous system and occurs in approximately 1 per every 1,000 live births in the United States. The condition may have other congenital anomalies associated with it, especially hydrocephalus. Assessment: 1. Spina Bifida Occulta a. Most patient have no symptoms. A dimple in the skin or hair growth may appear over the malformed vertebra. b. With growth, the child may develop foot weakness or bowel and bladder sphincter disturbances 2. Meningocele a. Physical examination shows a saclike outpouching in the spinal cord, usually in the midline b. Seldom evidence of weakness of the legs or lack of sphincter control. 3. Myelomeningocele a. Physical examination shows a round, raised, and poorly epithelialized area, usually in the lumbosacral area. Lesion may occur at any level of the spinal column. b. Loss of motor control and sensation below the level of the lesion. c. A low thoracic lesion may cause total flaccid paralysis below the waist. d. A small sacral lesion may cause only patchy spots of decreased sensation in the feet.

e. Contractures may occur in the ankles, knees of hips. f. Clubfoot commonly occurs, thought to be related position of paraplegic feet in the uterus. g. Urinary incontinence and retention and fecal incontinence and constipation occur because of impairment of sacral nerves.

General Assessment: - check site and level of lesion - motor and sensory levels - associated with hydrocephalus - associated with orthopedic deformity (hip dislocation)

Complications: Children with Spina Bifida experiences: - scoliosis - kyphosis - lower extremity contractures - hip dislocation In myelomeningocele, the spinal opening can be surgically repaired but the nerve damage is permanent resulting in paralysis of the lower limbs: - bladder and rectal dysfunction - damaged kidneys - hydrocephalus

Diagnostic Evaluation: 1. Prenatal detection is possible through amniocentesis and measurement of alphafetoprotein. This testing should be offered to all women at risk (women who are affected themselves or have had other affected children). 2. Diagnosis is primarily based on clinical manifestations. 3. CT Scan and MRI may be performed to further evaluate the brain and spinal cord and to evaluate hydrocephalus and any lesions 4. Urinary tract evaluations: urine analysis, urine culture, BUN, creatinine, ultrasound Prevention: - Adequate intake of folic acid before and during early pregnancy since people with spina bifida appears to have abnormal metabolism of folic acid - 1 month before conception and first 3 months of pregnancy

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. Collaborative Management: Therapeutic Interventions 1. A coordinated team approach will help maximize the physical and intellectual potential of each affected child. a. The team may include a neurologist, neurosurgeon, orthopedic surgeon, urologist, primary care provider, social worker, physical therapist, a variety of community-based hospital staff nurses, the child, and family. b. Numerous neurosurgical, orthopedic, and urologic procedures may be necessary to help the child achieve maximum potential. Surgical Interventions: 1. In meningocele and myelomeningocele, laminectomy and closure of an open lesion or removal of the sac usually can be done soon after birth. 2. Surgery is done to prevent further deteriorationof neural function, to minimize danger of rupture of sac and meningitis, to improve cosmetic effect, and to facilitate handling of infant. 3. In encephalocele, surgey during infancy repositions the bulging area back into the skull, removes the protrusions and corrects the deformities to relieve pressure.

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: Monitoring 1. Monitor and report immediately signs of infection. a. Oozing of fluid or pus from the sac b. Fever c. Irritability d. Seizure 2. Monitor urine elimination and report concentrated or foul-smelling urine, indicating a urinary tract infection (UTI). 3. Monitor signs of hydrocephalus and report immediately. a. Irritability b. Feeding difficulty, vomiting, decreased appetite c. Temperature fluctuation d. Decreased alterness e. Tense Fontanelle f. Increased head circumference 4. Frequently monitor temperature, pulse, respirations, color, and level of responsiveness postoperatively, based on the infant's stability.

Supportive Care
Preoperative care in Neonatal Period

1. Use prone positioning with hips only slightly flexed to decrease tension on the sac; check position at least once every hour. 2. Do not place diaper or any covering directly over the sac. 3. Observe the sac frequently for evidence of irritation or leakage of CSF. 4. Place padding between the infant's legs to maintain the hips abduction and to prevent or counteract subluxation. 5. Use a foam or fleece pad to reduce pressure of the mattress against the infant's skin. 6. Allow the infant's feet to hang freely over pads or matress edge to avoid aggravating 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 range of motion exercises for those muscles and joints that the infant does not use spontaneously. Avoid hip excercies because of common hip dislocation, unless otherwise recommended.

9. Avoid pressure on infants back during feeding by holding the with your elbow rotated to avoid touching the sac, or feeding while infant is lying on side or prone on your lap. Encourage parents to use these positions to provide infant stimulation and bonding. 10. Keep the buttocks and genitalia scupulously clean, infection of the sac is most commonly caused by contamination by urine and feces. a. Do not use diapers for 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 prevent contamination. 11. Apply sterile dressing over the sac only as directed and change frequently to prevent adhesion to sac and to maintain sterility. 12. To promote urinary elimination, use Crede's method to empty the bladder (unless contraindicated by vesicoureteral reflux), and teach parents the technique. Continue the procedure as long as urine can be manually expressed. 13. Straight catheterize the patient as needed. 14. Ensure fluid intake to dilute the urine.
Postoperative Care in Infancy and Childhood

1. Use an isolette or infant warmer to prevent temperature fluctuation. 2. 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. 3. Maintain hydration and nutritional intake. a. Administer IV Fluids as ordered, keep accurate intake and output log. b. Administer gavage feedings as ordered. c. Begin bottle feeding when infant responsive and tolerating feedings. 4. Be aware that children with spina bifida are susceptible to latex allergy. Symptoms include hives, itching, wheezing, and anaphylaxis. Incidence increases with time and may be related to repeated exposure. a. Limit or prevent direct contact of child with such products as blood pressure cuffs, tourniquets, tape, Foley catheters, gloves and IV tubing injection ports. 5. Teach parents that continence can usually be achieved with clean, intermittent self cathetetiration. a. Children can generally be taught to catheterize themselves by age 6 or 7. b. Parents can catheterize younger children. c. Red rubber catheters are used rather than latex catheters.
Education and Healthy Maintenance

1. Teach special techniques that may be required for holding and positioning, feeding, caring for the incision, emptying the bladder, and exercising the muscles.

2. Alert the parents for safety needs of the child with decrreased sensations, such as protection from prolonged pressure, risk of burns from bath water that is too warm, and avoidance of trauma from contact with sharp objects. 3. Reinforced that parents need to notify the health care provider for signs of associted problems such as hydrocephalus, meningitis, UTI, and latex sensitivity. 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 because of inactivity, so they should provide a low-fat, balance diet; control snacking; and encourage as much activity as possible.