Spina Bifida Pediatric Nursing Care Management and Study Guide

Spina bifida is a congenital neural tube defect that strikes the spine and spinal cord during early fetal development. With an open myelomeningocele the sac is your first priority: protect it from infection and pressure, keep urine and feces off it, and position the newborn prone until it is repaired. Past that, you are managing lifelong neurologic and musculoskeletal impairment and steadying a family that expected a healthy baby.

What is Spina Bifida?

Spina bifida is one of a group of birth defects called neural tube defects. It is caused by a defect in the neural arch, generally in the lumbosacral region: the posterior laminae of the vertebrae fail to close, leaving an opening through which the spinal meninges and spinal cord may protrude.

Classification

Spina bifida occulta is a bony defect without soft tissue involvement. Spina bifida with meningocele occurs when part of the spinal meninges protrudes through the bony defect and forms a cystic sac. In spina bifida with myelomeningocele, the spinal cord and meninges protrude, with nerve roots embedded in the wall of the cyst.

Pathophysiology

Neural tube defects result from a teratogenic process that causes failed closure and abnormal differentiation of the embryonic neural tube. In prenatal development, neuroectoderm thickens into the neural plate, which folds into a neural groove by the time somites appear. The groove deepens into the neural tube, and dorsal fusion begins centrally and extends cephalad and caudally, with the cephalad pole fusing at the 25th day. The ventricle becomes permeable at the 6th to 8th week of gestation, which does not proceed normally in spina bifida.

Statistics and Incidences

Incidence of spina bifida is estimated at 1 to 2 cases per 1000 population, with certain populations significantly higher by genetic predilection. Neural tube defects are the second most common birth defect after congenital heart defects, and myelomeningocele is the most common form. In the United States, about 1,500 infants are born with myelomeningocele each year. Birth incidence was 4.4 to 4.6 cases per 10,000 live births from 1983 to 1990, varying by region (higher on the East Coast than the West Coast, highest in Appalachia). Rates of myelomeningocele and other neural tube defects have declined since the late 20th century.

Average worldwide incidence is 1 case per 1000 births with marked geographic variation. Per 10,000 births, neural tube defects range from 0.9 in Canada and 0.7 in central France to 7.7 in the United Arab Emirates and 11.7 in South America. The highest rates are in parts of the British Isles, mainly Ireland and Wales, where 3 to 4 cases of myelomeningocele per 1000 population are reported; overall myelomeningocele incidence in the British Isles is 2 to 3.5 cases per 1000 live births. France, Norway, Hungary, Czechoslovakia, Yugoslavia, and Japan report a low prevalence of just 0.1 to 0.6 cases per 1000 live births. Per the CDC, US prevalence is higher in white and Hispanic populations (2 and 1.96 per 10,000 live births) than in the black population (1.74 per 10,000 live births). State and national surveillance from 1983 to 1990 found the birth prevalence of myelomeningocele slightly higher in females than males (1.2:1).

Causes

The etiology is usually multifactorial: genetic, racial, and environmental factors, with nutrition (particularly folic acid) central. Folate can reduce the incidence of neural tube defects by about 70% and lessen severity when they do occur. A woman who has had a baby with spina bifida carries a higher-than-normal risk of another affected baby (about 5%). Some medications for epilepsy or bipolar disorder are associated with higher risk of congenital defects including spina bifida. Women with diabetes are more likely to have an affected baby. Obese women, those with a BMI of 30 or more, carry higher risk, and the risk climbs as the BMI rises above 30.

Clinical Manifestations

Manifestations vary by type. If the opening is high on the spine, the legs are more likely to be completely paralyzed, with movement problems elsewhere. Neural tube problems harm brain development; the cortex, especially the frontal part, may not develop properly, producing cognitive problems. Type 2 Arnold-Chiari malformation, abnormal brain development involving the cerebellum, may affect language processing and physical coordination. A small birthmark, dimple, or tuft of hair may mark the skin over the spinal defect.

Assessment and Diagnostic Findings

Elevated maternal alpha fetoprotein in maternal serum and amniotic fluid indicates probable CNS abnormality. Fetal ultrasonography may show an incomplete neural tube. Diagnosis is made on clinical observation and examination. Further evaluation may include MRI, CT, and myelography.

Surgical Management

Many specialists are involved, especially with myelomeningocele. Surgery is required to close the open defect but may not be done immediately, depending on the surgeon's decision. Prenatal surgery takes place before the 26th week of pregnancy: surgeons expose the uterus, open it, and repair the baby's spinal cord. Babies with myelomeningocele may begin exercises preparing their legs for walking with braces or crutches when older. Cesarean birth may be part of treatment, since many babies with myelomeningocele are in a feet-first (breech) position.

Nursing Management

Nursing Assessment

Conduct a routine newborn exam with emphasis on neurologic impairment. Observe movement and response to stimuli in the lower extremities, carefully measure head circumference, and examine the fontanelles. Determine the family's knowledge and understanding of the defect and their attitude toward the birth of a newborn with such serious problems.

Nursing Diagnoses

Based on assessment data, the major nursing diagnoses are:

• Risk for infection related to vulnerability of the myelomeningocele sac.
• Risk for impaired skin integrity related to exposure to urine and feces.
• Risk for injury related to neuromuscular impairment.
• Compromised family coping related to perceived loss of the perfect newborn.
• Deficient knowledge of the family caregivers related to the complexity of caring for a newborn with serious neurologic and musculoskeletal defects.

Nursing Care Planning and Goals

The major goals are to prevent infection, maintain skin integrity, prevent trauma from disuse, and build family coping skills, education, and support.

Nursing Interventions

Prevent infection: monitor vital signs, neurologic signs, and behavior frequently, give prophylactic antibiotics as ordered, use routine aseptic technique, and cover the sac with a sterile dressing moistened in a warm sterile solution, changing it every 2 hours and covering it with a plastic protective layer. Promote skin integrity: place a protective barrier between the anus and the sac to prevent fecal contamination; diapering is not advisable with a low defect. Prevent contractures: newborns with spina bifida often have talipes equinovarus (clubfoot) and congenital hip dysplasia, so when the lower limbs lose motion, perform range-of-motion exercises, position the newborn with hips abducted and feet neutral, and massage and pad bony prominences regularly to protect them from irritation. Position the newborn prone so no pressure rests on the sac, and continue this after surgery until the site is well healed. Promote family coping: stay sensitive to their emotions, encourage open expression of feelings, give them privacy to mourn together, and encourage them to cuddle and touch the newborn using proper precautions for the defect. Provide teaching: give information about the defect, the newborn's current state, the proposed surgery, and followup care in small segments to aid comprehension; after surgery, teach the family to hold the newborn's head, neck, and chest slightly raised in one hand during feeding, and that stroking the cheeks stimulates sucking.

Evaluation

Expected outcomes are prevented infection, maintained skin integrity, prevented trauma from disuse, and increased family coping skills, education, and support.

Documentation Guidelines

Document individual risk factors including recent or current antibiotic therapy, signs and symptoms of infection, the characteristics of the lesion, causative and contributing factors, the caregiver's understanding of risks and safety concerns, current and past coping behaviors and emotional responses and available support systems, the plan of care, the teaching plan, responses to interventions and teaching, attainment or progress toward outcomes, modifications to the plan, and discharge needs, referrals, and who is responsible for actions to be taken.