Neural tube defects (also known as spina bifida or myelodysplasia)

Neural tube defects (also known as spina bifida or myelodysplasia) develop during first trimester of fetal development; defect can occur at any place along spinal canal.

1. Neural tube defects

2. Description Neural tube defects (also known as spina bifida or myelodysplasia ) develop during first trimester of fetal development; defect can occur at any place along spinal canal.

3. Etiology Etiology is unknown but maybe associated with maternal dietary folic acid deficiency. The degree of disability is determined by the location of defect and amount of spinal nerves encased in sac. The higher the defect, the greater the neurologic dysfunction.

4. Types of spina bifida 1. spina bifida occulta : posterior vertebral arches fail to fuse, but there is no herniation of spinal cord or meninges ; no loss of function. 2. Meningocele : posterior vertebral arches fail to fuse, and there is a saclike protrusion at some point along posterior vertebrae; sac does contain meninges and CSF but not spinal cord.

5. 3. Myelomeningocele: posterior vertebral arches fail to fuse; saclike herniation contains meninges , CSF, as well as portion of spinal cord or nerve roots; sometimes leakage of CSF occurs. 4. Encephalocele : brain and meninges herniate through defect in skull into a sac. 5. Anencephaly – absence of cerebral hemipheres . 6. microcephaly – fetal brain grows slowly

6. Assessment 1. prenatal diagnosis of open spinal defects can be determined by elevated levels of alpha-fetoprotein (AFP) in maternal serum and fluid obtained by amniocentesis; can also be assessed on prenatal ultrasound. Meningoceles , myelomeningoceles , and omphaloceles are obvious at birth ; SB occulta may have an associated abnormal tuft of hair, dimple, sinus tract or subcutaneous mass at the site.

7. During postnatal period, monitor for leakage of spinal fluid from sac as well as possible systemic or CNS infection. Assess degree of sensation at or below level of lesion; this can be evidenced by lack of movement or sensation in legs, and neurogenic (lacking innervation ) bladder or bowel. Measure head circumference since there is high risk of hydrocephalus

8. Priority Nursing Diagnosis Risk for infection Risk for impaired skin integrity Impaired urinary elimination Bowel incontinence/ Colonic constipation Impaired physical mobility

9. Planning and Implementation Collaborative management: the defect /sac is surgically repaired during first 48 hours after birth. Focus preoperative care in maintaining skin integrity of sac and keeping it free from infection; position infant on side or abdomen to attain this; keep sac moist with sterile, saline-soaked dressings; avoid contamination of sac area by urine and feces. Individuals with myelodysplasia have an increased incidence of latex allergies, monitor for this

10. Neurogenic bladder: frequent, straight catheterization is preferred method of management; maintain home schedule as much as possible. Neurogenic bowel: work with family to develop a bowel management plan using control of high-fiber diet, adequate fluid intake, and pattern for evacuation of bowels; in some cases, laxative and enemas are used as prescribed by physician.

11. Collaborate with physical therapy to develop modes of transport, such as using braces with crutches or wheelchair. Since areas with altered sensation are prone to skin breakdown, teach child and family to reposition frequently and inspect affected areas on a regular basis.

12. Medication therapy low-dose antibiotics may be prescribed to prevent UTIs

13. Child and family education Teach family about possibility of child developing hydrocephalus and signs and symptoms of increased ICP and what to do if changes develop. Since most children with neural tube defects (except for those with spina bifida occulta ) have some neurogenic bladder, teaching about clean intermittent straight catheterization is important; work with family to develop bowel management program also