Spina Bifida (Myelodysplasia)

  1. General information
    1. Failure of posterior vertebral arches to fuse during embryologic development
    2. Incidence: 2 in 1,000 infants in the U.S.
    3. Although actual cause is unknown, frequency of the defect is increased if a sibling has had a neural tube defect; radiation, viral, and environmental factors have been suggested as causative.
    4. Site of the defect varies
      1. Approximately 85% of the defects in the spine involve the lower thoracic lumbar or sacral area.
      2. Defects in the upper thoracic and cervical regions make up the remaining 15%.
  2. Types
    1. Spina bifida occulta
      1. Spinal cord and meninges remain in the normal anatomic position.
      2. Defect may not be visible, or may be identified by a dimple or a tuft of hair on the spine.
      3. Child is asymptomatic or may have slight neuromuscular deficit.
      4. No treatment needed if asymptomatic; otherwise treatment aimed at specific symptoms.
    2. Spina bifida cystica
      1. Meningocele
        1. sac (meninges) filled with spinal fluid protrudes through opening in spinal canal; sac is covered with thin skin
        2. no nerves in sac
        3. no motor or sensory loss
        4. good prognosis after surgery
      2. Myelomeningocele/meningomyelocele
        1. same as meningocele except there are spinal nerves in the sac (herniation of dura and meninges).
        2. child will have sensory/motor deficit below site of the lesion.
        3. 80% of these children have multiple handicaps.
  3. Medical management
    1. Surgery
      1. Closure of the sac within 48 hours of birth to prevent infection and preserve neural tissue
      2. Shunt procedure if accompanying hydrocephalus
      3. Orthopedic procedures to correct defects of hips, knees, or feet
    2. Drug therapy
      1. Antibiotics for prevention of infections.
      2. Anticholinergic drugs to increase bladder capacity and lower intravesicular pressure.
    3. Immobilization (casts, braces, traction) for defects of the hips, knees, or feet
  4. Assessment findings
    1. Examine the defect for size, level, tissue covering, and CSF leakage.
    2. Motor/sensory involvement may include
      1. Voluntary movement of lower extremities
      2. Withdrawal of lower extremities or crying after pinprick
      3. Paralysis of lower extremities
      4. Joint deformities
      5. Hydrocephalus
      6. Evaluate bowel and bladder function. Neurogenic bowel and bladder occur in up to 90% of the children.
    3. Diagnostic tests
      1. Prenatal
        1. ultrasound image of the pregnant uterus shows fetal spinal defect and sac
        2. amniocentesis: increased alphafetoprotein (AFP) level prior to 18th week of gestation
      2. Postpartal
        1. x-ray of spine shows vertebral defect; CT scan of skull may show hydrocephalus
        2. myelogram shows extent of neural defect
        3. encephalogram may show hydrocephalus
        4. urinalysis, culture and sensitivity (C&S) may identify organism and indicate appropriate antibacterial therapy
        5. BUN may be increased
        6. creatinine clearance rate may be decreased
  5. Nursing interventions
    1. Prevent trauma to the sac.
      1. Cover with sterile dressing soaked with normal saline.
      2. Position infant prone or side-lying.
      3. Keep the area free from contamination by urine or feces. A protective barrier drape may be necessary.
      4. Inspect the sac for intactness or signs of infection.
      5. Administer antibiotics as ordered.
    2. Prevent complications.
      1. Observe for signs of hydrocephalus, meningitis, joint deformities.
      2. Clean intermittent urinary catheterization to manage neurogenic bladder.
      3. Administer medications to prevent urinary complications as ordered.
      4. Perform passive ROM exercises to lower extremities.
    3. Provide adequate nutrition: adapt diet and feeding techniques according to the child's position.
    4. Provide sensory stimulation.
      1. Adjust objects for visual stimulation according to child's position.
      2. Provide stimulation for other senses.
    5. Provide emotional support to parents/family.
    6. Provide client teaching and discharge planning to parents concerning
      1. Wound care
      2. Physical therapy, range of motion exercises
      3. Signs of complications
      4. Medication regimen: schedule, dosage, effects, and side effects
      5. Feeding, diapering, positioning
      6. Availability of appropriate support groups/community agencies/genetic counseling