Neural Tube Defects

The nurse is preparing to admit a 2-year-old child with spina bifida occulta. What clinical manifestations of spina bifida occulta should the nurse expect to observe? (Select all that apply.) a. Dark tufts of hair b. Skin depression or dimple c. Port-wine angiomatous nevi d. Soft, subcutaneous lipomas e. Bladder and sphincter paralysis

A, B, C, D Clinical manifestations of spina bifida occulta include dark tufts of hair; skin depression or dimple; port-wine angiomatous nevi; and soft, subcutaneous lipomas. Bladder and sphincter paralysis are present with spina bifida cystica but not occulta

A 12-year-old child with Guillain-Barr syndrome (GBS) is admitted to the pediatric intensive care unit. She tells you that yesterday her legs were weak and that this morning she was unable to walk. After the nurse determines the current level of paralysis, which should the next priority assessment be? a. Swallowing ability b. Parental involvement c. Level of consciousness d. Antecedent viral infections

A. Assessment of swallowing is essential. Both pharyngeal involvement and respiratory function are usually involved at the same time. The child may require ventilatory support. The inability to swallow also contributes to aspiration pneumonia. Parental involvement is important after the physiologic assessment is complete. The child is answering questions and describing the onset of the illness, which demonstrates she is alert and oriented. Information regarding antecedent viral infections can be obtained after the child is assessed and stabilized.

The nurse is caring for a neonate born with a myelomeningocele. Surgery to repair the defect is scheduled the next day. What is the most appropriate way to position and feed this neonate? a. Prone with the head turned to the side b. On the side c. Supine in an infant carrier d. Supine, with defect supported with rolled blankets

A. The prone position with the head turned to the side for feeding is the optimum position for the infant. It protects the spinal sac and allows the infant to be fed without trauma. The side-lying position is avoided preoperatively. It can place tension on the sac and affect hip dysplasia if present. The infant should not be placed in a supine position.

MATH TIME!! The health care provider has prescribed neostigmine (Prostigmin) 0.04 mg/kg/per dose SC q 4 to 6 hrs PRN for a child with myasthenia gravis. The child weighs 77 lb. The nurse is preparing to administer a dose. Calculate the dose the nurse should administer in milligrams. Record your answer below using one decimal place.

ANS: 1.4 The correct calculation is: 77 lb/2.2 kg = 35 kg Dose of Prostigmin is 0.04 mg/kg/dose 0.04 mg 35 = 1.4 mg

The health care provider has prescribed oxybutynin (Ditropan) 0.2 mg/kg/day divided bid for a child with myelomeningocele. The child weighs 33 lb. The nurse is preparing to administer the 0900 dose. Calculate the dose the nurse should administer in milligrams. Record your answer below using one decimal place.

ANS: 1.5 The correct calculation is: 33 lb/2.2 kg = 15 kg Dose of Ditropan is 0.2 mg/kg/day divided bid 0.2 mg 15 = 3 mg 3 mg/2 = 1.5 mg

The nurse is preparing to admit a 5-year-old child with a lower motor neuron syndrome. What clinical manifestations of a lower motor neuron syndrome should the nurse expect to observe? (Select all that apply.) a. Loss of hair b. Babinski reflex present c. Skin and tissue changes d. Marked atrophy of atonic muscle e. Hyperreflexia with tendon reflexes exaggerated

ANS: A, C, D Clinical manifestations of a lower motor neuron syndrome include loss of hair, skin and tissue changes, and marked atrophy of atonic muscle. Babinski reflex present and hyperreflexia with tendon reflexes exaggerated are manifestations of an upper motor neuron syndrome.

The nurse is preparing to admit a 7-year-old child with an upper motor neuron syndrome. What clinical manifestations of an upper motor neuron syndrome should the nurse expect to observe? (Select all that apply.) a. No flexor spasms b. Babinski reflex present c. No wasting of muscle mass d. Marked atrophy of atonic muscle e. Hyperreflexia with tendon reflexes exaggerated

ANS: B, C, E Clinical manifestations of an upper motor neuron syndrome include Babinski reflex present, no wasting of muscle mass, and hyperreflexia with tendon reflexes exaggerated. No flexor spasms and marked atrophy of atonic muscle are manifestations of a lower motor neuron syndrome.

When a child develops latex allergy, which food may also cause an allergic reaction? a. Yeast b. Wheat c. Peanuts d. Bananas

ANS: D There are cross-reactions between allergies to latex and to a number of foods such as bananas, avocados, kiwi, and chestnuts. Although yeast, wheat, and peanuts are potential allergens, currently they are not known to cross-react with latex allergy.

The nurse is preparing to admit a 5-year-old with spina bifida cystica that was below the second lumbar vertebra. What clinical manifestations of spina bifida cystica below the second lumbar vertebra should the nurse expect to observe? (Select all that apply.) a. No motor impairment b. Lack of bowel control c. Soft, subcutaneous lipomas d. Flaccid, partial paralysis of lower extremities e. Overflow incontinence with constant dribbling of urine

B, D, E The clinical manifestations of spina bifida cystica below the second lumbar vertebra include lack of bowel control, flaccid, partial paralysis of lower extremities, and overflow incontinence with constant dribbling of urine. No motor impairment occurs with spina bifida cystica that was below the third lumbar vertebra, and soft, subcutaneous lipomas occur with spina bifida occulta.

A goal for children with spina bifida is to reduce the chance of allergy development. What is a priority nursing intervention? a. Recommend allergy testing. b. Provide a latex-free environment. c. Use only powder-free latex gloves. d. Limit use of latex products as much as possible.

B. A latex-free environment is the goal. This includes eliminating the use of latex gloves and other medical devices containing latex. Allergy testing would provide information about whether the allergy has developed. It will not reduce the chances of developing the allergy. Although powder-free latex gloves are less allergenic, latex should not be used. Limiting the use of latex products is one component of providing a latex-free environment, but latex products should not be used.

The nurse is caring for a family whose infant was just born with anencephaly. What is the most important nursing intervention? a. Implement measures to facilitate the attachment process. b. Help the family cope with the birth of an infant with a fatal defect. c. Prepare the family for extensive surgical procedures that will be needed. d. Provide emotional support so the family can adjust to the birth of an infant with problems.

B. Anencephaly is the most serious neural tube defect. The infants have an intact brainstem and, if born alive, may be able to maintain vital functions for a few hours to several weeks. The family requires emotional support and counseling to cope with the birth of an infant with a fatal defect. The parents should be encouraged to hold their infant and provide comfort measures. This facilitates the grieving process because the infant has a limited life expectancy. Infants with anencephaly do not have cerebral hemispheres. There is no surgical correction available for this defect. Emotional support is needed as the family adjusts to the birth of a child who has a fatal defect.

The most important nursing intervention when caring for an infant with myelomeningocele in the preoperative stage is which? a. Take vital signs every hour. b. Place the infant on the side to decrease pressure on the spinal sac. c. Watch for signs that might indicate developing hydrocephalus. d. Apply a heat lamp to facilitate drying and toughening of the sac.

B. The spinal sac is protected from damage until surgery is performed. Early surgical closure is recommended to prevent local trauma and infection. Monitoring vital signs and watching for signs that might indicate developing hydrocephalus are important interventions, but preventing trauma to the sac is a priority. The sac is kept moist until surgical intervention is done.

A recommendation to prevent neural tube defects (NTDs) is the supplementation of what? a. Vitamin A throughout pregnancy b. Folic acid for all women of childbearing age c. Folic acid during the first and second trimesters of pregnancy d. Multivitamin preparations as soon as pregnancy is suspected

B. The widespread use of folic acid among women of childbearing age has decreased the incidence NTDs. In the United States, the rates of NTDs have declined from 1.3 per 1000 births in 1990 to 0.3 per 1000 after the introduction of mandatory folic acid supplementation in food in 1998. Vitamin A is not related to the prevention of NTDs. Folic acid supplementation is recommended for the preconceptual period, as well as during the pregnancy. The NTD is a failure of neural tube closure during early development, the first 3 to 5 weeks.

What most accurately describes bowel function in children born with a myelomeningocele? a. Incontinence cannot be prevented. b. Enemas and laxatives are contraindicated. c. Some degree of fecal continence can usually be achieved. d. Colostomy is usually required by the time the child reaches adolescence.

C. With a combination of dietary modification, regular toilet habits, and prevention of constipation and impaction, some degree of fecal continence can usually be achieved. Incontinence can be minimized with the development of a regular bowel training program. A surgical intervention can assist with continence. Enemas and laxatives are part of a bowel training program. Colostomies are not indicated in children with myelomeningocele.

What refers to a hernial protrusion of a saclike cyst of meninges, spinal fluid, and a portion of the spinal cord with its nerves through a defect in the vertebral column? a. Rachischisis b. Meningocele c. Encephalocele d. Myelomeningocele

D. A myelomeningocele has a visible defect with an external saclike protrusion, containing meninges, spinal fluid, and nerves. Rachischisis is a fissure in the spinal column that leaves the meninges and the spinal cord exposed. Meningocele is a hernial protrusion of a saclike cyst of meninges with spinal fluid but no neural elements. Encephalocele is a herniation of brain and meninges through a defect in the skull, producing a fluid-filled sac.

The nurse is caring for a child with myasthenia gravis (MG). What health care prescription should the nurse verify before administering? a. Ceftizoxime (Cefizox) b. Cefotaxime (Claforan) c. Ceftriaxone (Rocephin) d. Garamycin (gentamicin)

D. Avoid aminoglycoside antibiotics such as gentamicin because they potentiate MG symptoms. Cefizox, Claforan, and Rocephin are cephalosporin antibiotics.

Neuropathic bladder disorders are common among children with which disorder? a. Plagiocephaly b. Meningocele c. Craniosynostosis d. Myelomeningocele

D. Myelomeningocele is one of the most common causes of neuropathic bladder dysfunction among children. Plagiocephaly is the flattening of a side of the child's head. This is not associated with neuropathic bladder. Children with meningocele usually do not have neuropathic bladder. Craniosynostosis is the premature closure of one or more cranial sutures. It is not associated with neuropathic bladder.

An 8-year-old child is hospitalized with infectious polyneuritis (Guillain-Barr syndrome [GBS]). When explaining this disease process to the parents, what should the nurse consider? a. Paralysis is progressive with little hope for recovery. b. Disease is inherited as an autosomal, sex-linked, recessive gene. c. Disease results from an apparently toxic reaction to certain medications. d. Muscle strength slowly returns, and most children recover.

D. Recovery usually begins within 2 to 3 weeks, and most patients regain full muscle strength. The paralysis is progressive with proximal muscle weakness occurring before distal weakness. The recovery of muscle strength occurs in the reverse order of onset of paralysis. Most individuals regain full muscle strength. Better outcomes are associated with younger ages. GBS is an immune-mediated disease often associated with a number of viral or bacterial infections or the administration of vaccines.

A woman who is 6 weeks pregnant tells the nurse that she is worried that, even though she is taking folic acid supplements, the baby might have spina bifida because of a family history. The nurses response should be based on what? a. Prenatal detection is not possible yet. b. There is no genetic basis for the defect. c. Chromosome studies done on amniotic fluid can diagnose the defect prenatally. d. Open neural tube defects (NTDs) result in elevated concentrations of a-fetoprotein in amniotic fluid.

D. Ultrasound scanning and measurement of a-fetoprotein may indicate the presence of anencephaly or myelomeningocele. The optimum time for performing this analyzing is between 16 and 18 weeks. Prenatal diagnosis is possible through amniocentesis. A multifactorial origin is suspected, including drugs, radiation, maternal malnutrition, chemicals, and possibly a genetic mutation. Chromosome abnormalities are not present in NTDs.