Chapter 21: The Newborn at Risk: Congenital Disorders

A nurse is caring for a child with complex esophageal atresia who will be undergoing surgery for repair. What comment by the parents indicates further teaching is required?

"After this surgery is done tomorrow, my baby will be able to eat and drink." The newborn will need IV fluids to maintain optimal hydration. The first stage of surgery may involve a gastrostomy and a method of draining the proximal esophageal pouch. A chest tube is inserted to drain chest fluids. If the repair is complex, surgery may need to be done in stages.

The nursing instructor is discussing congenital heart disease with a group of students. Which statement indicates that students need further teaching?

"The ductus arteriosus carries deoxygenated blood from the aorta to the pulmonary artery during fetal life." The students need additional teaching if they report that the ductus arteriosus takes deoxygenated blood from the aorta to the pulmonary artery during fetal life. The ductus arteriosus carries oxygenated blood from the pulmonary artery to the aorta during fetal life. Blood returns to the heart from the inferior vena cava, and oxygenated blood travels to the body through the aorta.

The nurse is caring for a newborn client newly diagnosed with developmental dysplasia of the hip (DDH). Which response by the nurse educates the parents on the correct plan of treatment for this diagnosis?

"Treatment will begin immediately." Developmental dysplasia of the hip (DDH) is a congenital newborn condition that requires immediate intervention. The development of the acetabulum of the hip is defective, and it may or may not be dislocated. Treatment of the defect and dislocated hips involves positioning the hip into a flexed, abducted (externally rotated) position to attempt to press the femur into the acetabulum. This involves splints and halters as the first line of treatment. Treatment should not be delayed. Surgery and casts are typically not used as the first line of treatment.

A newborn boy is diagnosed with esophageal atresia and tracheoesophageal fistula. After the nurse provides preoperative teaching, which statement indicates that the parents need additional teaching?

"We can probably start feeding him with the bottle about a day after the surgery." Preoperatively, the newborn is at risk for pneumonitis due to aspiration of food and secretions. Antibiotics are typically given until the anastomosis is proven intact and patent. Oral feedings are usually started within a week after surgery once the esophageal anastomosis is proven to be intact and patent. Proper position with elevation of the head is important for the newborn with esophageal atresia and tracheoesophageal fistula because he is at risk for aspiration of food and secretions. Using a pacifier to provide nonnutritive sucking helps to meet the newborn's need to suck.

An older infant is scheduled to have a cleft palate repair. The mother asks if she will still be able to breast-feed the baby during the postoperative phase. What is the best response by the nurse?

"You will not be able to breast-feed immediately after, but you can pump and feed the child with a cup." For an infant who has had a palate repair, no nipples, spoons, or straws are permitted; only a drinking glass or a cup is recommended. A favorite cup from home may be reassuring to the older infant.

The nurse is reviewing the medical record of the antepartum client with an abnormal alpha-fetoprotein test. The mother is distraught and states, "How bad can it be?" The nurse is correct to describe which?

A type of spina bifida The alpha-fetoprotein test is an indicator of a neural tube defect indicating a form of spina bifida. Spina bifida is a bony defect that occurs is various forms and can produce a varied level of disability (ranging from no disability to paralysis). This test is not an indicator of a cardiac deficit, a cleft lip and palate, or a kidney disorder.

Immediately after birth, the nurse is caring for a newborn with a myelomeningocele. What intervention should the nurse provide to prevent drying out of the sac to avoid damage?

Apply a sterile dressing moistened in a warm, sterile saline solution. Until surgery is performed, the sac must be covered with a sterile dressing moistened in a warm, sterile solution (often saline). The nurse should change this dressing every 2 hours and not allow it to dry to avoid damage to the covering of the sac.

Which health care provider assessment technique does the nurse anticipate being used to determine developmental dysplasia of the hip (DDH) on a newborn?

Barlow sign and Ortolani click The nurse anticipates that a Barlow sign and Ortolani assessment will be done by an experienced health care provider when the newborn is in the nursery. This includes range of motion of the hip. Leg kicks and visual inspection are not helpful in determining congenital hip dysplasia.

The parent has brought a 2-year-old to the public clinic for immunizations. The nurse documents the following characteristics: A duck waddle gait Shortened extremity Asymmetry of the gluteal folds Protruding abdomen The nurse then refers the toddler to the health care provider for potential diagnosis of which?

Developmental dysplasia of the hip (DDH) Developmental dysplasia of the hip (DDH) exhibits signs of asymmetry of the gluteal folds, lordosis, swayback, protruding abdomen, shortened extremity, and a duck-like waddle. Congenital talipes equinovarus is clubfoot. Scoliosis is a curvature of the spine. Muscular dystrophy is a chronic degenerative muscular condition.

Which assessment findings are most prominent in the infant with Tetralogy of Fallot and significant pulmonary stenosis?

Dyspnea on limited exertion, fatigue, cyanosis The infant with Tetralogy of Fallot and significant pulmonary stenosis exhibits prominent signs of dyspnea, fatigue and cyanosis. Other symptoms include feeding difficulties and poor weight gain, retarded growth and development and breathlessness. Irregular heartrate, dry mucous membranes, nausea and decreased muscle tone may be present in some form but are not the prominent signs.

Which nursing suggestion is most helpful in preventing symptoms from a 3-month-old's hiatal hernia?

Feed in an upright position The nursing suggestion which is most helpful is to feed the infant in an upright position and maintain that position after the feeding. The upright posture is a noninvasive, easy suggestion to try without a health care provider's order. An increased amount of feeding may aggravate the symptoms. Adding cereal to the feeding and use of a hypoallergenic formula would need approval from the health care provider.

From which pair of metabolic disorders must the nurse instruct the parents to eliminate breast and cow's milk from the diet?

Galactosemia and phenylketonuria Both phenylketonuria and galactosemia are hereditary disorders in which the body cannot have milk. Maple syrup urine disease is an inborn error of metabolism of the branched chain amino acid. Congenital hypothyroidism is an error with the thyroid gland.

The nurse is caring for a neonate in the newborn nursery with clubfoot (congenital talipes equinovarus). If nonsurgical treatment is chosen, which nursing action is anticipated?

Holding feet/ankles in position for casting The nurse is caring for a neonate with clubfoot (congenital talipes equinovarus). If nonsurgical treatment is chosen, the nurse action anticipated would be holding the feet and ankles in the position determined by the health care provider for casting. Serial casting over time will provide the appropriate correction. Ace wraps will not maintain the intended position. Corrective shoes alone do not change the position of the feet and ankle. A Dennis Browne splint includes shoes with the splint.

The parent reports that the health care provider said that the infant had a hernia but she can't remember which type. When recalling what the health care provider said, the parent said that a surgeon will repair it soon and there is no problem with the testes. Which hernia type is anticipated?

Inguinal hernia An inguinal hernia occurs primarily in males and allows the intestine to slip into the inguinal canal, resulting in swelling. If the intestine becomes trapped and circulation is impaired, surgery is indicated within a short period of time. The diaphragmatic hernia has implications with the respiratory system. An umbilical hernia typically spontaneously closes by age 3. A hiatal hernia produces digestive issues.

The nursing student is describing a protrusion of the spinal cord and the meninges. The nursing instructor realizes that the student is correctly describing which neural tube defect?

Myelomeningocele Myelomeningocele is a defect in the neural tube that includes the spinal cord and the meninges in a cyst. Spina bifid a occult does not have a cyst, meningocele does not include the spinal cord and a spinal cyst does not include the spinal cord or the meninges.

The nurse is caring for a neonate with epispadias. In which location will the nurse assess the anomaly?

On the dorsal end of the penis The nurse would assess the epispadias on the dorsal (top) surface of the penis. This condition often occurs with exstrophy of the bladder. The other options are incorrect locations.

A 5-month-old is having a cleft lip/palate repair. What games could the nurse play with the child to provide comfort and diversion postoperatively while the baby is in elbow restraints?

Peek-a-boo Playing peek-a-boo and other infant games will help to comfort and entertain the infant in restraints; however, "patty cake" does not work well with an infant in elbow restraints. Blowing a pinwheel will stress the suture line.

A nurse is caring for a newborn client who is diagnosed with myelomeningocele. Which nursing intervention would protect the newborn from injury?

Place the newborn in a prone or lateral position. The nurse should place the newborn in a prone or lateral position to keep pressure off the spinal sac and avoid newborn injury. Parents should be allowed to hold the stable infant with assistance. The spinal sac should be kept covered and moist until surgery to avoid rupture. Collecting urine decreases the risk of urine stasis, which is sometimes common in spinal defects.

A nurse is caring for a newborn with a repaired cleft lip. What intervention can the nurse provide to facilitate drainage of mucus and secretions to prevent aspiration?

Position the child on the side. To facilitate drainage of mucus and secretions, the nurse should position the infant on the side, never on the abdomen, after a cleft lip repair.

The practical nurse is assisting the registered nurse with a neonate following ventriculoperitoneal shunting (VP shunt) related to hydrocephalus. Which assessment findings are communicated with the registered nurse immediately? Select all that apply.

Temperature of 100.7ºF (38.2ºC) Anterior fontanelles noted as raised The registered nurse must assess the neonate's condition following surgery. The LPN/LVN provides follow-up care and will notify the RN immediately if signs of infection of increased intracranial pressure are noted. An elevated temperature is noted as is a raised fontanelle. Though not bulging, the LPN/LVN would refer to the RN and note past documentation. A decreased head circumference, 116 beats/min heart rate, incision slightly pink with no drainage and an alert, pleasant neonate are normal assessment findings for this stage of recovery.

The nurse is caring for a neonate with an exstrophy of the bladder. When the nurse is planning care, which client goal is the priority?

The client will be free from infection. The highest priority goal is that the client will be free from infection. This client has open mucosa of the bladder and a developed tract that leads to the bladder and then the kidney. Nursing intervention must include frequent vital signs; inspection of the site; observation for drainage, color and clarity of urine in diaper; and frequent urinalysis as ordered until surgical correction. Bonding is always a goal when caring for a newborn and family. Having an adequate urine output is an appropriate goal. Due to the sensitive nature of the mucosa, it is important for the neonate to not experience discomfort, particularly when the area is being cleansed.

When caring for parents whose neonate is newly diagnosed with a congenital disorder, which parents would be referred to pediatric hospice services?

The parent with a neonate diagnosed with maple syrup urine disease The parent with a neonate diagnosed with maple syrup urine disease (MSUD) would be referred to pediatric hospice services due to the prognosis of the disease. The disease is rapidly progressive and often fatal. The parents with a neonate diagnosed with Down syndrome and hydrocephalus will grieve the loss of the perfect child. The nurse must provide much education and support throughout the child's life. Most neonates with spina bifida occulta are asymptomatic. Instruction is needed for understanding the disease process.

The nurse is reinforcing discharge teaching with the mother of an infant who is being discharged prior to having a required blood test done. The nurse explains to this mother that she needs to bring the newborn back to check the infant's phenylalanine level. Which statement is most accurately related to this blood test?

The test is done after the newborn has ingested protein. As soon as the newborn with phenylketonuria begins to take milk, phenylalanine builds up in the blood serum to as much as 20 times the normal level. This build-up occurs so quickly that increased levels of phenylalanine appear in the blood after only 1 or 2 days of ingestion of milk.

The nurse is educating the parents of a neonate with Down syndrome regarding nutrition. Which provides the biggest challenge in feeding the neonate?

Thick, fissured tongue When feeding the neonate with Down syndrome, the biggest challenge includes the status of the tongue. In infancy, the child's large tongue and poor muscle control contribute to difficulty with breastfeeding or ingesting formula; this can cause great problems when it comes to beginning table foods. The majority of children with Down syndrome feed themselves. Shortness of the head, brachycephaly, and decreased gastric motility do not impact the ability for feeding.

Which congenital condition is an immediate emergency requiring notification of the health care provider?

Tracheoesophageal fistula The congenital condition which is a medical emergency is a tracheoesophageal fistula. This condition can lead to respiratory distress and pneumonitis. Hypospadias is the urethra opening terminating on the ventral surface of the penis, instead of the tip. Cleft palate is the opening in the roof of the mouth. An atrial septal defect is an opening between the right and left atrial. Hypospadias, cleft palate, and an atrial septal defect may be surgically repaired but are not immediate emergencies.

The nurse is observing the perineal care of a 2-year-old in a hip spica cast. For which caregiver actions will the nurse provide additional instruction? Select all that apply.

Uses the tips of the fingers to handle the cast Applies powder to the perineal area after a bowel movement The nurse would provide additional instruction related to handling the cast with the palms of the hands instead of tips of the fingers. Also, the caregiver would be advised to refrain from using powders as powders can build up and irritate the skin. The other options demonstrate appropriate care.

In the infant diagnosed with spina bifida with myelomeningocele, the infant will likely have:

a partial to complete paralysis in the lower extremities. In spina bifida with myelomeningocele, there is a protrusion of the spinal cord and the meninges, with nerve roots embedded in the wall of the cyst. The effects of this defect vary in severity from sensory loss/partial paralysis below the lesion to complete flaccid paralysis of all muscles below the lesion.

When examining a newborn for developmental dysplasia of the hip (DDH), which motion would the newborn's hip be unable to accomplish?

abduction Infants with shallow acetabulums are unable to abduct their hips.

In the hour immediately following the birth of an infant with a physical challenge, what is a nursing care priority? Select all that apply.

determining the infant's immediate physiologic needs promoting bonding between parents and the newborn Nursing priorities include determining physiologic needs and promoting bonding. Outlining long-term implications, referring to genetics, and exploring behaviors that may have contributed to the condition can all be delayed until a later time.

A pregnant client asks the nurse at what point in pregnancy the fetal heart is most susceptible to damage during development. The nurse correctly explains the period as:

during the first 8 weeks of pregnancy when it is forming. The fetal heart develops between the third and eighth week of pregnancy. Teratogenic effects would be most detrimental during this time.

A nurse in the newborn nursery has noticed that an infant is frothing and appears to have excessive drooling. Further assessment reveals that the baby has episodes of respiratory distress with choking and cyanosis. What disorder should the nurse suspect based on these findings?

esophageal atresia Any swallowed mucus or fluid enters the blind pouch of the esophagus when a newborn suffers from esophageal atresia. The newborn with this disorder will have frothing, excessive drooling, and periods of respiratory distress with choking and cyanosis. If this happens, no feedings should be given until the newborn has been examined.

A nursing student has read that cleft lip is diagnosed at birth based on inspection of physical appearance and that cleft palate is diagnosed by which method?

feeling the palate with a gloved finger or using a tongue blade Diagnosis of cleft palate is made at birth with close inspection of the newborn's palate. To be certain that a cleft palate is not missed, the examiner must insert a gloved finger into the newborn's mouth to feel the palate to determine that it is intact. The other tests cannot confirm a cleft palate.

Congenital myelomeningocele is commonly associated with which condition?

hydrocephalus A myelomeningocele is commonly associated with hydrocephalus or excessive cerebrospinal fluid (CSF) within the cranial cavity. Microcephaly is associated with maternal exposure to cytomegalovirus (CMV) or rubella. Anencephaly is a different type of neural tube defect. Cranial suture overlap may occur with vaginal birth, but it is not associated with myelomeningocele.

For which condition would the nurse commonly assess in an infant following surgery for a myelomeningocele?

hydrocephalus Surgery includes removing a portion of the meninges; without the surface to absorb cerebral spinal fluid, hydrocephalus can result.

A newborn is diagnosed with congenital hypothyroidism prior to discharge from the hospital. What medication does the nurse anticipate administering to the newborn?

levothyroxine The thyroid hormone must be replaced as soon as the diagnosis is made. Levothyroxine sodium, a synthetic thyroid hormone replacement, is the drug most commonly used.

When planning preoperative care for a newborn with a cleft lip and palate, the nurse would plan interventions for which major need?

nutrition An infant with a cleft lip is unable to suck effectively, so obtaining adequate nutrition is a major concern.

It would be best to place an infant with a myelomeningocele in which position prior to surgery?

on the stomach (prone) Placing the infant prone prevents direct trauma to the lesion and reduces the chance that feces will contaminate the lesion.

A mother is inspecting her newborn and notices the baby has a sixth finger. The nurse would explain that this condition is called:

polydactyly. Polydactyly is the presence of one or more additional fingers or toes. Syndactyly is when two fingers or toes are fused. Webbing is the result of fusing of two fingers or toes. Genu varum is a knee disorder.

A nurse is providing preoperative care to a female newborn client with the congenital abnormality myelomeningocele. Which intervention is the priority?

preventing infection A congenital condition of the newborn with a spinal deformity puts the newborn at risk for infection. A myelomeningocele is a fluid-filled sac on the spine that includes part of the spinal cord defect and the meninges. This cyst on the outside of the newborn requires surgical intervention. Although nutrition, GI function, and motor function are all important to the health of the newborn, the spinal and meninges defect puts the newborn at high risk for infection.

What treatment can the nurse anticipate assisting with for a newborn with clubfoot (congenital talipes equinovarus)?

serial casting Treatment for clubfoot (congenital talipes equinovarus) starts during the neonatal period. Correction can usually be accomplished by manipulation and bandaging or by application of a cast. Casts are changed frequently to provide gradual, atraumatic correction—every few days for the first several weeks.

A nurse is conducting an assessment of a 13-month-old infant. The mother notes that the infant cannot pull oneself into a standing position. Which assessment should the nurse conduct to gather more information to report to the health care provider?

symmetry of gluteal skin folds assessment By assessing for symmetry of gluteal skin folds the nurse would be assessing for signs of developmental dysplasia of the hip. It is expected that by about 10 months of age infants can pull themselves into a standing position.

The nurse is providing education to the parents of an infant who was just diagnosed with transposition of the great arteries. The parents ask, "Which vessels were involved?" The nurse is correct to educate about:

the aorta and pulmonary artery. The nurse is correct to educate that in the congenital condition known as transposition of the great arteries, it is the aorta and the pulmonary artery that are reversed. In this condition, the aorta rises from the right ventricle and the pulmonary artery arises from the left.

The nurse is caring for a newborn with hydrocephalus. To protect the newborn from injury in the postoperative period, the nurse should position the head:

turned away from the operative site. In the early postoperative period, the infant's head should be placed turned away from the operative site to promote comfort until the physician instructs otherwise. Trendelenberg would facilitate additional fluid accumulation, infants should not be placed on pillows and if turned toward the operative site additional pain and fluid accumulation would result.

A student nurse is learning about congenital heart defects in newborns and correctly identifies which of the following to be the most common intracardiac defect?

ventricular septal defect Ventricular septal defect is the most common intracardiac defect. It consists of an abnormal opening in the septum between the two ventricles.