NEURAL TUBE DEFECTS (SPINA BIFIDA, MENINGOCELE, MYELOMENINGOCELE) Neural tube defects include defects of the spinal cord

that occur as a result of failure of neural tube closure in embryonic development. They may involve the entire length of the neural tube or may be restricted to a small area. Defects involving failure of the osseous (bony) spine to close are called spina bifida (SB); SB occulta occurs in lumbosacral area (L5 and S1), and there is no obvious external opening; SB cystica refers to a visible defect with an external saclike protrusion. The two major forms of SB cystica are meningocele, which encases meninges and spinal fluid but no neural elements and myelomeningocele (or meningomyelocele), which contains meninges, spinal fluid, and nerves. Meningocele is not associated with neurologic deficit, which occurs in varying degrees in myelomeningocele. With myelomeningocele, the degree of neurologic dysfunction depends on where the sac protrudes through the vertebrae, the anatomic level of the defect, and the amount of nerve tissue involved. Most myelomeningoceles involve the lumbar or lumbosacral area. Hydrocephalus is a frequently associated anomaly in 80% to 90% of children. Pathophysiology Most authorities believe that the primary defect in NTDs is a failure of neural tube closure during early development (the first 3 to 5 weeks) of the embryo. There is evidence implicating a multifactorial etiology including drugs, radiation, maternal malnutrition, chemicals, and possibly a genetic mutation in folate pathways in some cases, which may result in abnormal development. A genetic component in the development of SB is seen in some cases; myelomeningocele may occur in association with syndromes such as trisomy 18, PHAVER syndrome and Meckel-Gruber syndrome. Myelomeningocele may result in musculoskeletal problems of the lower extremities affecting ambulation; in addition, neurologic deficits may be evident in bowel and bladder function. Cognitive function is normal in such children. Clinical Signs and Symptoms SB occulta: no defect noted except by palpation of vertebra Meningocele: visible evidence of protruding sac on spinal cord (lumbar) Myelomeningocele: visible sac protruding on lumbar or lumbosacral area

Diagnostic Evaluation MRI, CT scan, and myelography Bladder function studies may be performed.

Treatment Meningocele and myelomeningocele: protection of sac; moist saline gauze; avoid laying on sac; early surgical closure in most cases Evaluation of lower extremity involvement, urinary function, bowel function and interventions to preserve function, enhance mobilization, and prevent complications Long-term care involves promotion of optimal functioning in regards to ambulation and mobilization, and urinary and bowel continence. Physical therapy

Nursing Care Management Focused on myelomeningocele Identification of defect Protect sac: moist saline gauze; avoid laying on sac. Assist parents and support; encourage close contact; explain treatments. Pre- and postoperative care; monitoring vital signs; protection and care of incision; prevent infection. Routine newborn care: temperature maintenance; may be fed postoperatively Observe for development of hydrocephalus postoperatively: increasing head circumference. Physical therapy to prevent lower extremity contractures and muscle atrophy Observe for complications such as urinary stasis and UTI; perianal skin care for stool incontinence.

Discharge teaching Patient and Family Teaching Holding and feeding (postop) Observation for complications: urinary stasis (may require clean intermittent catheterization at home); head circumference growth; poor feeding; perianal skin care for loose stools Encourage follow-up visits with specialists (urologist, orthopedist, neurosurgeon, primary practitioner). Access to multidisciplinary team (spina bifida clinic) as applicable to promote optimal development.