Chapter 32 THE NEWBORN AT RISK: CONDITIONS PRESENT AT BIRTH 917

CONGENITAL ANOMALIES: IDENTIFICATION AND CARE IN NEWBORN PERIOD

Congenital Anomaly Nursing Assessments Nursing Goals and Interventions
Congenital hydrocephalus
Choanal atresia
Cleft lip
Cleft palate
Tracheoesophageal fistula
(type 3)
Assess presence of hydrocephalus: Measure and plot occipital-frontal
baseline measurements; then measure head circumference once a day.
Check fontanelles for bulging and sutures for widening.
Assist with head ultrasound and transillumination.
Maintain skin integrity: Change position frequently.
Clean skin creases after feeding or vomiting.
Use sheepskin pillow under head.
Postoperatively, position head off operative site.
Watch for signs of infection.
Assess patency of nares: Listen for breath sounds while holding babys mouth
closed and alternately compressing each nostril.
Assist with passing feeding tube to confirm diagnosis.
Maintain respiratory function: Assist with taping airway in mouth to prevent
respiratory distress.
Position with head elevated to improve air exchange.
Provide nutrition: Feed with special nipple.
Burp frequently (increased tendency to swallow air and reflex vomiting).
Clean cleft with sterile water (to prevent crusting on cleft prior to repair).
Support parental coping: Assist parents with grief over loss of idealized baby.
Encourage verbalization of their feelings about visible defect.
Provide role model in interacting with infant. (Parents internalize others
responses to their newborn.)
(At left) Unilateral cleft lip with cleft abnormality involving both hard and soft palates.
Prevent aspiration/infection: Place prone or in side-lying position to facilitate
drainage.
Suction nasopharyngeal cavity (to prevent aspiration or airway obstruction).
During newborn period feed in upright position with head and chest tilted
slightly backward (to aid swallowing and discourage aspiration).
Provide nutrition: Feed with special nipple that fills cleft and allows sucking.
Also decreases chance of aspiration through nasal cavity.
Clean mouth with water after feedings.
Burp after each ounce (tend to swallow large amounts of air).
Thicken formula to provide extra calories.
Plot weight gain patterns to assess adequacy of diet.
Provide parental support: Refer parents to community agencies and support
groups.
Encourage verbalization of frustrations because feeding process is long and
frustrating.
Praise all parental efforts.
Encourage parents to seek prompt treatment for upper respiratory infection
(URI) and teach them ways to decrease URI.
Maintain respiratory status and prevent aspiration:
Withhold feeding until esophageal patency is determined.
Quickly assess patency before putting to breast in birth area.
Place on low intermittent suction to control saliva and mucus (to prevent
aspiration pneumonia).
Place in warmed, humidified incubator (liquefies secretions, facilitating removal).
Elevate head of bed 2040 degrees (to prevent reflux of gastric juices).
Keep quiet (crying causes air to pass through fistula and to distend intestines,
causing respiratory embarrassment).
Maintain fluid and electrolyte balance: Give fluids to replace esophageal
drainage and maintain hydration.
Provide parent education: Explain staged repairprovision of gastrostomy
and ligation of fistula, then repair of atresia.
Keep parents informed; clarify and reinforce physicians explanations
regarding malformation, surgical repair, preoperative and postoperative care,
and prognosis (knowledge enhances feelings of self-worth).
Enlarged head
Enlarged or full fontanelles
Split or widened sutures
Setting sun eyes
Head circumference 90% on growth
chart
Occlusion of posterior nares
Cyanosis and retractions at rest
Snorting respirations
Difficulty breathing during feeding
Obstruction by thick mucus
Unilateral or bilateral visible defect
May involve external nares, nasal
cartilage, nasal septum, and alveolar
process
Flattening or depression of midfacial
contour
Fissure connecting oral and nasal cavity
May involve uvula and soft palate
May extend forward to nostril involving
hard palate and maxillary alveolar ridge
Difficulty in sucking
Expulsion of formula through nose
History of maternal hydramnios
Excessive mucous secretions
Constant drooling
Abdominal distention beginning soon after
birth
Periodic choking and cyanotic episodes
Immediate regurgitation of feeding
Clinical symptoms of aspiration
pneumonia (tachypnea, retractions,
rhonchi, decreased breath sounds,
cyanotic spells)
Failure to pass nasogastric tube
(continued)
918 SIX THE NEWBORN
CONTINUED
Congenital Anomaly Nursing Assessments Nursing Goals and Interventions
Tracheoesophageal fistula
(type 3) continued
Diaphragmatic hernia
Involve parents in care of infant and in planning for future; facilitate touch and
eye contact (to dispel feelings of inadequacy, increase self-esteem and self-
worth, and promote incorporation of infant into family).
(At left) The most frequently seen type of congenital tracheoesophageal fistula and
esophageal atresia.
Nurse should never ventilate with bag and mask O
2
because the stomach and
intestines will become distended with air, further compressing the lungs.
Maintain respiratory status: Immediately administer oxygen. May need to be
intubated and ventilated.
Initiate gastric decompression.
Place in high semi-Fowlers position (to use gravity to keep abdominal organs
pressure off diaphragm).
Turn to affected side to allow unaffected lung expansion.
Carry out interventions to alleviate respiratory and metabolic acidosis.
Assess for increased secretions around suction tube (denotes possible
obstruction).
Aspirate and irrigate tube with air or sterile water.
(At left) Diaphragmatic hernia. Note compression of the
lung by the intestine on the affected side.
Difficulty initiating respirations
Gasping respirations with nasal flaring
and chest retraction
Barrel chest and scaphoid abdomen
Asymmetric chest expansion
Breath sounds may be absent
Usually on left side
Heart sounds displaced to right
Spasmodic attacks of cyanosis and
difficulty in feeding
Bowel sounds may be heard in thoracic
cavity
Esophagus
Trachea
Lung
Esophagus
Stomach
Bowel through
opening in
diaphragm
Diaphragm
Liver
Chapter 32 THE NEWBORN AT RISK: CONDITIONS PRESENT AT BIRTH 919
CONTINUED
Congenital Anomaly Nursing Assessments Nursing Goals and Interventions
Myelomeningocele
Omphalocele
Gastroschisis
Imperforate anus, congenital
dislocated hip, and clubfoot
Prevent trauma and infection.
Position on abdomen or on side and restrain (to prevent pressure and trauma to sac).
Meticulously clean buttocks and genitals after each voiding and defecation (to prevent
contamination of sac and decrease possibility of infection).
May put protective covering over sac (to prevent rupture and drying).
Observe sac for oozing of fluid or pus.
Cred bladder (apply downward pressure on bladder with thumbs, moving urine toward
the urethra) as ordered to prevent urinary stasis.
Assess amount of sensation and movement below defect.
Observe for complications.
Obtain occipital-frontal circumference baseline measurements; then measure head
circumference once a day (to detect hydrocephalus).
Check fontanelle for bulging.
(At left) Newborn with lumbar myelomeningocele.
SOURCE: Courtesy Dr Paul Winchester.
Maintain hydration and temperature.
Provide D
5
LR and albumin for hypovolemia.
Place infant in sterile bag up to and covering defect.
Cover sac with moistened sterile gauze, and place plastic wrap over dressing
(to prevent rupture of sac and infection).
Initiate gastric decompression by insertion of nasogastric tube attached to
low suction (to prevent distention of lower bowel and impairment of blood
flow).
Prevent infection and trauma to defect.
Position to prevent trauma to defect.
Administer broad-spectrum antibiotics.
Maintain hydration and temperature.
Prevent trauma and infection to defect.
Provide D
5
LR and albumin for hypovolemia.
Place infant in sterile bag up to cord and covering defect.
Initiate gastric decompression by insertion of nasogastric tube attached to
low suction.
Administer broad-spectrum antibiotics.
Identify defect and initiate appropriate referral early.
Saclike cyst containing meninges, spinal
cord, and nerve roots in thoracic and/or
lumbar area
Myelomeningocele directly connects to
subarachnoid space so hydrocephalus
often associated
No response or varying response to
sensation below level of sac
May have constant dribbling of urine
Incontinence or retention of stool
Anal opening may be flaccid
Herniation of abdominal contents into
base of umbilical cord
May have an enclosed transparent sac
covering
Lateral defect in abdominal wall allowing
viscera outside the body to the left of an
intact umbilical cord. No sac covering.
Associated with intestinal atresia,
malrotation
See discussion in Chapter 29, Anus and
Extremities
Source: Courtesy of Nancy Houck, RNC, BSN, NNP.
may suffer withdrawal. In addition, the drugs the mother in-
gested may be teratogenic, resulting in congenital anomalies.
Alcohol Dependence
The fetal alcohol syndrome (FAS) includes a series of mal-
formations (identified shortly) frequently found in infants
exposed to alcohol in utero. It has been estimated that com-
plete FAS occurs in 5.2 live births per 1000 (AAP Commit-
tee on Substance Abuse and Committee on Children with
Disabilities, 2000). FAS rates are increased among Native
Americans and Alaska natives and those of low economic sta-
tus. Fetal alcohol effects (FAE), or alcohol-related birth
defects (ARBD), are usually determined only by a positive
maternal drinking history and cognitive difficulties and usu-
ally do not exhibit the classic facial dysmorphology (Rosen &
Bateman, 2002). The new diagnostic categories for FAS take
into consideration the various clinical manifestations of FAS,
the social and family environment, and, if available, the ma-
ternal alcohol history.