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Congenital Anomaly

Nursing Assessments

Nursing Goals and Interventions

Congenital hydrocephalus

Enlarged head
Enlarged or full fontanelles
Split or widened sutures
“Setting sun” eyes
Head circumference  90% on growth

Choanal atresia

Occlusion of posterior nares
Cyanosis and retractions at rest
Snorting respirations
Difficulty breathing during feeding
Obstruction by thick mucus

Cleft lip

Unilateral or bilateral visible defect
May involve external nares, nasal
cartilage, nasal septum, and alveolar
Flattening or depression of midfacial

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 baby’s 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.)

Cleft palate

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

Tracheoesophageal fistula
(type 3)

History of maternal hydramnios
Excessive mucous secretions
Constant drooling
Abdominal distention beginning soon after
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

(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
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
Encourage verbalization of frustrations because feeding process is long and
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 20–40 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 repair—provision of gastrostomy
and ligation of fistula, then repair of atresia.
Keep parents informed; clarify and reinforce physician’s explanations
regarding malformation, surgical repair, preoperative and postoperative care,
and prognosis (knowledge enhances feelings of self-worth).





Congenital Anomaly

Nursing Assessments

Nursing Goals and Interventions
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 selfworth, and promote incorporation of infant into family).

Tracheoesophageal fistula
(type 3) continued


(At left) The most frequently seen type of congenital tracheoesophageal fistula and
esophageal atresia.

Diaphragmatic hernia

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

Nurse should never ventilate with bag and mask O2 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-Fowler’s 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
Aspirate and irrigate tube with air or sterile water.



Bowel through
opening in

(At left) Diaphragmatic hernia. Note compression of the
lung by the intestine on the affected side.



Congenital Anomaly

Nursing Assessments

Nursing Goals and Interventions


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

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.
Credé 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.


Herniation of abdominal contents into
base of umbilical cord
May have an enclosed transparent sac


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,

Imperforate anus, congenital
dislocated hip, and clubfoot

See discussion in Chapter 29, Anus and

Maintain hydration and temperature.
Provide D5LR 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
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 D5LR 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.

Source: Courtesy of Nancy Houck, RNC, BSN, NNP.

may suffer withdrawal. In addition, the drugs the mother ingested may be teratogenic, resulting in congenital anomalies.

Alcohol Dependence
The fetal alcohol syndrome (FAS) includes a series of malformations (identified shortly) frequently found in infants
exposed to alcohol in utero. It has been estimated that complete FAS occurs in 5.2 live births per 1000 (AAP Committee 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 status. Fetal alcohol effects (FAE), or alcohol-related birth
defects (ARBD), are usually determined only by a positive
maternal drinking history and cognitive difficulties and usually 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 maternal alcohol history.