Suggested Answers to Assignments, Chapter 21, The Newborn at Risk: Congenital

Disorders

Written Assignments Learning Objective(s)

1. Students’ answers should include the following: 4
• A failure of the maxillary and premaxillary processes to

fuse during fetal development can cause a cleft lip on

one or both sides of the lip or a cleft palate, in which the

tissue in the roof of the mouth does not fuse properly.

Surgery is a major part of the treatment of either

condition. Nursing care focuses on maintaining adequate

nutrition, increasing family coping, and reducing the

parents' anxiety and guilt. After surgery, nursing care

focuses on preventing aspiration, improving respiration,

maintaining adequate fluid volume and nutritional

requirements, relieving pain, preventing injury and

infection to the surgical site, and promoting normal

growth and development.

2. Students’ answers should include the following: 7

 A diaphragmatic hernia occurs when abdominal

organs are displaced into the left chest through an

opening in the diaphragm. A hiatal hernia occurs if

the cardiac portion of the stomach slides into the

area above the diaphragm. A rare occurrence, an

 omphalocele is seen when the abdominal contents

protrude through the umbilical cord and form a sac

lying on the abdomen. If the end of the umbilical

cord does not close completely and a portion of the

intestine protrudes through the opening, an umbilical

hernia forms. Inguinal hernias occur mostly in males

when a part of the intestine slips into the inguinal

canal.

 Most hernias can be repaired surgically.

3. Students’ answers should include the following: 11

• Phenylketonuria (PKU) is a recessive hereditary defect

of metabolism that, if untreated, causes severe

intellectual disability. PKU is diagnosed with the

Guthrie inhibition assay test to detect phenylalanine

levels in the blood. Dietary treatment using a formula

and diet low in phenylalanine is started and continued as

the child gets older. Galactosemia is a hereditary

metabolic disorder in which the enzyme necessary to

convert galactose into glucose is missing. Galactose is

omitted from the diet to treat the disorder. Congenital

hypothyroidism is detected by performing tests for

triiodothyronine (T3) and thyroxine (T4) levels to

determine thyroid function. The thyroid hormone must

be replaced to treat the disorder. Maple syrup urine

 disease (MSUD) is an inborn error of metabolism, which

is autosomal recessive in inheritance. It is rapidly

progressive and often fatal.

• If PKU, congenital hypothyroidism, and galactosemia

are not treated, the newborn often has severe intellectual

disability.

Group Assignments Learning Objective(s)

1. Students’ answers should include the following: 5
• Esophageal atresia (EA) is the absence of a normal

opening in or the abnormal closure of the esophagus. A

tracheoesophageal fistula occurs when the upper, or

proximal, end of the esophagus ends in a blind pouch, and

the lower, or distal, segment from the stomach is

connected to the trachea by a fistulous tract. Early signs of

EA include frothing and excessive drooling and periods of

respiratory distress with choking and cyanosis. The

newborn with tracheoesophageal fistula is at risk for

aspiration, pneumonitis, and respiratory distress. Surgical

correction of the defect is necessary.

2. Students’ answers should include the following: 6, 8, 9, 13

• Hypospadias is a congenital condition in which the urethra

terminates on the ventral (underside) surface of the penis,

 instead of at the tip. Epispadias occurs when the opening

of the urethra is on the dorsal (top) surface of the penis. In

the child with exstrophy of the bladder, the anterior

surface of the urinary bladder lies open on the lower

abdomen; surgical closure of the bladder is preferred

within the first 48 hours of life. Ambiguous genitalia is a

condition in which the external sexual organs are either

incompletely or abnormally formed, and it may not be

possible to determine by observation at birth if the child is

a male or a female.

Clinical Assignments Learning Objective(s)

1. Students’ answers should include the following: 1
• Spina bifida is caused when the spinal vertebrae fail to

close and an opening is left where the spinal cord or

meninges may protrude. In spina bifida occulta, soft tissue

is not involved, and only a dimple in the skin may be seen.

In spina bifida with meningocele, the spinal meninges

protrude through and form a sac, and in spina bifida with

myelomeningocele, both the spinal cord and meninges

protrude. Myelomeningocele is the most difficult type to

treat because of the concern of complete paralysis below

the lesion.
2. Students’ answers should include the following: 2
Noncommunicating hydrocephalus occurs when there is an
obstruction in the circulation of CSF. With communicating
hydrocephalus, absorption of CSF is defective. The most
obvious symptom of hydrocephalus is the rapid increase in
head circumference. Ventriculoperitoneal shunting (VP shunt)
drains the CSF from the brain into the peritoneal cavity.
Ventriculoatrial shunting drains the CSF into the heart.
Nursing care focuses on preventing injury.
3. Students’ answers should include the following: 10

 Signs and symptoms of developmental dysplasia of

the hip include asymmetry of the gluteal skin folds,
limited abduction of the affected hip, and apparent
shortening of the femur. To treat hip dislocation, the
femur is manipulated and a brace applied. A hip spica
cast may be used after an open reduction, if necessary.
Nursing care focuses on relieving pain and discomfort,
maintaining skin integrity, promoting growth and
development, and increasing family knowledge.

4. Students’ answers should include the following: 12

• Down syndrome is sometimes called trisomy 21 because

of the three-chromosome pattern seen on the 21st pair of

chromosomes. Signs and symptoms seen in children

include brachycephaly (shortness of head); slowed

growth; slanted (almond-shaped) eyes; short, flattened

nose; thick tongue; dry, cracked, fissured skin; dry and

coarse hair; short hands with an incurved fifth finger;

single horizontal palm crease (simian line); wide space

between the first and second toes; lax muscle tone; heart

and eye anomalies; and a greater susceptibility to

 leukemia.

Web Assignment Learning Objective

1. Students’ answers should include the following: 3
• Ventricular septal defects allow the blood to pass from the

left to the right ventricle; in the atrial septal defect the

blood flows from the left to the right atria. With a patent

ductus arteriosus, the blood is shunted from the aorta into

the pulmonary artery. When coarctation of the aorta

occurs, there is a narrowing of the aortic arch and an

obstruction of blood flow.

• Tetralogy of Fallot is a group of heart defects including

pulmonary stenosis, ventricular septal defect, overriding

aorta, and right ventricular hypertrophy. The child with

tetralogy of Fallot has cyanosis and low oxygen

saturation. The severe and usually fatal defect,

transposition of the great arteries, causes cyanosis and

occurs because the aorta arises from the right ventricle

instead of the left, and the pulmonary artery arises from

the left ventricle instead of the right.