JUSTINE C.
LAGERA
2BSN1 NCM109
(Based on Maternal Child Nursing Care by Lowdermilk)
THE HIGH RISK NEWBORN  o Other trauma requires some degree of intervention; few
 A high risk neonate can be defined as a newborn, regardless
of Gestational age or birth weight, who has a greater-than-
average Chance of morbidity or mortality because of conditions or
circumstances associated with birth and the adjustment to extra
uterine existence.  When the newborn is born:
 High risk infants are most often classified according to birth weight,
gestational age, and predominant pathophysiologic problems.  
Classification According To Size
• Low-birth-weight (LBW) infant—Infant whose birth
weight is less than 2500 g (5 lbs 8 oz), regardless of
gestational age
• Very low–birth-weight (VLBW) infant—Infant whose
birth weight is less than 1500 g (3 lbs 5 oz)
• Extremely low–birth-weight (ELBW) infant—Infant
whose birth weight is less than 1000 g (2 lb 3 oz)
• Appropriate-for-gestational-age (AGA) infant—
Infant whose weight falls between the 10th and 90th
percentiles on intrauterine growth curves
• Small-for-date (SFD) or small-for-gestational-age
(SGA) infant— An infant whose rate of intrauterine
growth was slowed and whose birth weight falls below the
10th percentile on intrauterine growth curves
• Intrauterine growth restriction (IUGR) —Found in
infants whose intrauterine growth is restricted (sometimes
used as a more descriptive term for SGA infants)
• Symmetric IUGR—Growth restriction in which the
weight, length, and head circumference are all affected
• Asymmetric IUGR—Growth restriction in which the
head circumference remains within normal parameters
while the birth weight falls below the 10th percentile
• Large-for-gestational-age (LGA) infant—Infant
whose birth weight falls above the 90th percentile on
intrauterine growth charts
Classification According to Gestational Age
• Preterm (premature) infant—Infant born before
completion of 37 weeks of gestation, regardless of birth
weight
• Full-term infant—Infant born between the beginning of
the 38 weeks and the completion of the 42 weeks of
gestation, regardless of birth weight
• Late-preterm infant—Infant born between 34 0/7 and
36 6/7weeks of gestation, regardless of birth weight*
BIRTH INJURIES
 Birth trauma (injury) is physical injury sustained by a neonate
during labor and birth.
 Most birth injuries are avoidable, especially with careful
assessment of risk factors and appropriate planning of the birth
 FETAL ULTRASONOGRAPHY
o Allows antepartum diagnosis of certain fetal conditions that
may be treated in utero or shortly after birth.
 Elective cesarean birth can be chosen for some pregnancies to
prevent significant birth injury.
 Many injuries are minor and resolve readily in the neonatal
period without treatment.
are serious enough to be fatal.
CARE MANAGEMENT
 The nurse makes a rapid inspection and physical assessment to
determine whether there are any lifethreatening conditions that
require immediate medical or surgical attention
 A comprehensive BOX 25-1 assessment of the new born is
physical
performed after the parents have had the opportunity to interact
with their new baby.
Evidence of some birth injuries may not be apparent at the initial
examination, assessment continues during each contact with the
neonate.
A. SKELETAL INJURIES
 The newborn’s immature, flexible skull can withstand a great
degree of deformation (molding) before fracture results.
 Considerable force is required to fracture it.
Two types of skull fractures
1. Linear
2. epressed
 If an artery lying in a groove on the undersurface of the skull is
torn as a result of the fracture
o Increased intracranial pressure (ICP) follows.
 Unless a blood vessel is involved, linear fractures, which account
for 70% of all fractures for this age-group, heal without special
treatment.
 The soft skull may become indented without laceration of either
the skin or the dural membrane
o These depressed fractures, or Ping-Pong ball indentations,
may occur during difficult births from pressure of the head
on the bony pelvis.
o can also occur as a result of injudicious application of
forceps
 The clavicle is the bone most often fractured during birth.
Generally the break is in the middle third of the bone
 Dystocia, particularly shoulder impaction, may be the predisposing
problem.
Often present on affected part are:
 Limited arm motion,
 crepitus over the bone, and
 absence of the Moro reflex
 no accepted treatment for fractured clavicle of the new-born exists,
 The humerus and femur are other bones that may be fractured
during a difficult birth.
 o Fractures in newborns generally heal rapidly.
 Immobilization is accomplished with:
o slings,
o splints,
o swaddling, and
o other devices.
MANAGEMENT/TEACHING
 The parents need support in handling these infants because they
often are afraid of hurting them
 Encouraged to practice handling, changing, and feeding the
affected neonate under the guidance of nursery personnel.
o increases their confidence and knowledge and facilitates
attachment
 A plan for follow-up therapy is developed with the parents so the
times and arrangements for therapy are acceptable to them.

PERIPHERAL NERVOUS SYSTEM INJURIES

-it involves the nerves, they are the nerves that come down and make
your hands and arms work. They leave your spinal cord
Plexus injury
Results from forces that alter the normal position and relationship of the
arm, shoulder, and neck
Erb palsy (Erb Duchenne paralysis)
 Caused by damage to the upper plexus and usually results from
a stretching or pulling away of the shoulder from the head such
as might occur with shoulder dystocia or a difficult vertex or
breech birth
CLINICAL MANIFESTATIONS OF ERB PALSY
 Related to the paralysis of the affected extremity and muscles:
 arm hangs limp alongside the body
 shoulder and arm are adducted and rotated internally
 elbow is extended, and the forearm is pronated with the wrist
and fingers flexed
 a grasp reflex may be present because finger and wrist
movement remains normal
Klumpke palsy,
 results from severe stretching of the upper extremity while the
trunk is relatively less mobile
 the muscles of the hand are paralyzed, with consequent wrist
drop and relaxed fingers
 In a third and more severe form of brachial palsy, the entire
arm is paralyzed and hangs limp and motionless at the side.
 The Moro reflex is absent on the affected side for all of the
forms of brachial palsy
Treatment of the affected arm is aimed at preventing contractures of
the paralyzed muscles and maintaining correct placement of the humeral
head within the glenoid fossa of the scapula.
Complete recovery from stretched nerves usually takes 3 to 6 months.
Complete recovery from stretched nerves usually takes 3 to 6 months
Nursing care of the newborn with brachial palsy
 concerned primarily with proper positioning of the affected arm
 affected arm should be abducted 90 degrees with external shoulder
rotation, forearm supination, and extension at the wrist with the
palm facing the infant’s face
 Passive range-of-motion exercises of the shoulder, wrist, elbow,
and fingers are initiated in the latter part of the first week.
 Wrist flexion contractures may be prevented with the use of a wrist
splint with padding in the fist
 In dressing infants, Undressing begins with the unaffected arm, and
redressing begins with the affected arm to prevent unnecessary
manipulation and stress on the paralyzed muscles
 Instruct parents to use the football position when holding the infant
 avoid picking the child up from under the axillae or pulling on the
arms.
Pressure on the facial nerve (cranial nerve VII)
- during birth may result in injury to it
- 
clinical manifestations
- loss of movement on the affected side such as an inability to
completely close the eye
- drooping of the corner of the mouth, and absence of wrinkling
of the forehead and nasolabial fold
Facial palsy
- most noticeable when the infant cries
- mouth is drawn to the unaffected side
- wrinkles are deeper on the normal side
- the eye on the involved side remains open
- Often the condition is temporary, resolving within hours or days
of birth.
- Permanent paralysis is rare unless the nerve fibers were torn, in
which case surgical intervention may be necessary
Nursing care of the infant with facial nerve paralysis
- Helping the infant suck and the mother with feeding
techniques.
- The infant may require gavage feeding to prevent aspiration
- Breastfeeding is not contraindicated but the mother needs
additional assistance to help the infant grasp and compress the
areolar area.
- If the eyelid on the affected side does not close completely,
artificial tears can be instilled daily to prevent drying of the
conjunctiva, sclera, and cornea.
- The eyelid is often taped shut to prevent accidental injury.
- If eye care is needed at home, the parents are taught the
procedure for administering eyedrops before the infant is
discharged
Phrenic nerve paralysis
 results in diaphragmatic paralysis as demonstrated by
ultrasonography, which shows paradoxical chest movement and an
elevated diaphragm
 The injury sometimes occurs in conjunction with brachial palsy
 Respiratory distress is the most common and important sign of
injury.
o Injury to the phrenic nerve is usually unilateral, the lung
on the affected side does not expand, and respiratory
efforts are ineffectual.
 The infant is positioned on the affected side to facilitate maximum
expansion of the uninvolved lung.
 Breathing is primarily thoracic; cyanosis, tachypnea, or complete
respiratory failure may be seen. Pneumonia and atelectasis on the
affected side may also occur
 Breathing is primarily thoracic; cyanosis, tachypnea, or complete
respiratory failure may be seen. Pneumonia and atelectasis on the
affected side may also occur
Neurologic Injuries
 Neurologic injury in newborn infants is common with the increased
survival of low-birth-weight and very low–birth-weight infants
 lower the gestational age, the higher the risk for certain
neurologic injuries
Such infants are particularly vulnerable to ischemic injury caused
by variable (both increased and decreased) cerebral blood flow
subsequent to asphyxia;
 and preterm infants, with a fragile cerebrovascular network, are
highly prone to periventricular or intraventricular hemorrhage.
 Fragility and increased permeability of capillaries and prolonged
prothrombin time predispose preterm infants to trauma when
delicate structures are subjected to the forces of labor
The more common cerebral complications and nursing care Neonatal neutrophils are present in term infants but have decreased
functional capabilities; response to infections is sluggish.
Phagocytosis is less efficient. Serum complement levels are low in
term infants and even lower in the preterm infant;
serum complement (C1 through C6) is involved in immunologic
reactions, some of which kill or lyse bacteria and enhance
phagocytosis.
The gut mucosal barrier is initially immature in both term and
preterm infants;
This barrier is enhanced by the ingestion of human colostrum,
which contains antiinfective properties. Dysmaturity seen with
intrauterine growth restriction (IUGR) and preterm and postdate
birth further compromises the immune system of the neonate.
RISK FACTOR FOR NEONATAL SEPSIS
SOURCE RISK FACTORS
MATERNAL Low socioeconomic status, Poor prenatal care,
Poor nutrition, Substance abuse
INTRAPART Premature rupture of membranes, Maternal fever,
UM Chorioamnionitis, Prolonged labor, Rupture of
membranes >18 hr, Premature labor Maternal
urinary tract infection
NEONTAL Twin or multiple gestation, Male, Birth asphyxia,
Meconium aspiration, Congenital anomalies of
skin or mucous membranes, Galactosemia,
Absence of spleen, Low birth weight or preterm
birth, Malnourishment, Prolonged
hospitalization, Invasive procedures

Neonatal infections may be acquired in utero, at birth or shortly

thereafter, and as a health care–associated infection (HAI).
Neonatal bacterial infection is classified into two patterns according to
the time of presentation.
1. Early-onset or congenital sepsis
usually manifests within 24 to 48 hours of birth
progresses more rapidly than later-onset infection, and carries a
mortality rate as high as 50%.
acquired in the perinatal period;
can occur from direct contact with organisms from the maternal
GI and genitourinary tracts
Escherichia coli and group B streptococcus (GBS) (most
common infecting organism)
o GBS is an extremely virulent organism in neonates,
with a high (50%) death rate in affected infants
o Other bacteria noted to cause early-onset infection
include Haemophilus influenzae, Citrobacter and
Enterobacter organisms, coagulase-negative
staphylococci, and Streptococcus viridans
o Other pathogens that are harbored in the vagina and
The highest incidence of abnormal neurologic findings occurs in very may infect the infant include gonococci, Candida
low–birth-weight (VLBW) infants and those with intracranial albicans, herpes simplex virus (HSV) type 2, and
haemorrhage chlamydia.
Major neurologic problems such as Early-onset sepsis is associated with a history of obstetric events
1. cerebral palsy, such as preterm birth, prolonged rupture of membranes (more
2. seizures, and than 18 hours), maternal fever during labor, and
3. hydrocephalus are chorioamnionitis. Early-onset infection is also inversely related
 usually diagnosed in the first 2 years of life. to infant birth weight
Less severe deficits such as 2. Late-onset sepsis,
1. learning disorders, occurring approximately at 7 to 30 days of age,
2. attention deficit hyperactivity disorder (ADHD), and considered primarily to be an infection acquired in the hospital or
3. fine- and gross-motor incoordination community
 may not be diagnosed until preschool or even school age. the offending organisms are usually staphylococci, Klebsiella
Cerebral palsy is one of the most common neurologic deficits in organisms, enterococci, E. coli, and Pseudomonas or Candida
survivors of preterm birth. species
Bacterial invasion can occur through sites such as the umbilical
stump; the skin; mucous membranes of the eye, nose, pharynx,
and ear; and internal systems such as the respiratory, nervous,
urinary, and GI systems.
Perinatally acquired infections may cause
o miscarriage,
o stillbirth,
o intrauterine infection,
o congenital malformations, and
o acute neonatal disease.
It is important to recognize the manifestations of infections in the
neonatal period to be able to treat the acute infection,
o prevent HAIs in other infants, and
o anticipate effects on the infant’s subsequent growth
and development.
NEONATAL INFECTIONS Fungal infections
SEPSIS greatest concern in the immunocompromised or preterm infant
the presence of microorganisms or their toxins in the blood or other Occasionally fungal infections such as thrush are found in
tissues otherwise healthy term infants
one of the most significant causes of neonatal morbidity and Septicemia - a generalized infection in the bloodstream
mortality. Pneumonia - most common form of neonatal infection, is one of the
Maternal immunoglobulin M (IgM) does not cross the placenta. leading causes of perinatal death
IgG levels in term infants are equal to maternal levels; however, in Bacterial meningitis - occurs in approximately 0.2 to 0.4 cases per
preterm infants the amount of IgG is directly proportional to 1000 live births, with a higher rate in preterm infants
gestational age. Gastroenteritis – is sporadic, depending on epidemic outbreaks. Local
IgA and IgM require time to reach optimum levels after birth infections such as conjunctivitis and thrush occur commonly
 Review the prenatal record for risk factors associated with
infection and the signs and symptoms suggestive of infection.
o Maternal vaginal or perineal infection may be transmitted
directly to the infant during passage through the birth canal.
 Psychosocial history and history of sexually transmitted infections
(STIs) may indicate possible human immunodeficiency virus
(HIV), hepatitis B virus (HBV), herpes (type 2), or
cytomegalovirus (CMV) infection.\
 Perinatal events should also be reviewed
o Premature rupture of membranes (PROM) may be caused by
maternal or intrauterine infection
o Ascending infection may occur after prolonged PROM,
prolonged labor, or intrauterine fetal monitoring. In some
cases infection may occur with intact membranes or
contribute to early rupture.
 A maternal history of fever during labor or the presence of foul-
smelling amniotic fluid may also indicate infection
 Antibiotic therapy initiated during labor should be noted.
 The neonate’s
o gestational age,
o maturity,
o birth weight, and
o gender affect the incidence of infection
 Sepsis occurs about twice as often and results in a higher mortality
in male than in female infants.
 Assess the neonate for
o respiratory distress,
o skin abscesses,
o petechial rashes, and
o other indications of infection.
During the postnatal period
 note the time of onset of suspicious clinical signs.
o Onset within the first 48 hours of life is more often
associated with prenatal or perinatal predisposing factors;
onset after 2 or 3 days more often reflects an HAI.
clinical signs of neonatal sepsis
 lack of specificity.
 The nonspecific signs include l
o ethargy, poor feeding,
o poor weight gain, and
o irritability
 The nurse or parent may simply note that the infant is not doing as
well as before
 Differential diagnosis may be difficult because signs of sepsis are
similar to signs of noninfectious neonatal problems such as
hypoglycemia and respiratory distress
CLINICAL MANIFESTATIONS OF SEPSIS
SYSTEM SIGNS
RESPIRATORY Apnea, Tachypnea Grunting, nasal flaring
Retractions, Decreased oxygen saturation,
Metabolic acidosis
CARDIOVASCUL Decreased cardiac output, Tachycardia,
AR Bradycardia, Hypotension, Decreased
perfusion
CENTRAL Temperature instability, Lethargy,
NERVOUS Hypotonia, Irritability, seizures
GASTROINTEST Feeding intolerance (decreased suck strength
INAL and intake; increasing residuals) Abdominal
distention Vomiting, diarrhea
INTEGUMENTA Jaundice, Pallor, Petechiae, Mottling
RY
Laboratory studies
 Specimens for cultures include blood, cerebrospinal fluid (CSF),
stool, and urine
 A complete blood cell count with differential is performed to
determine the presence of bacterial infection or increased or
decreased white blood cell count (the latter is an ominous sign).
 The total neutrophil count, immature-to-total (I/T) neutrophil ratio,
absolute neutrophil count, platelet count, procalcitonin, and C-
reactive protein may be used to determine the presence of sepsis
Breastfeeding
 (medically stable infants) or feeding the newborn expressed
breast milk from the mother is encouraged
 Breast milk provides protective mechanisms.
 Colostrum contains IgA, which offers protection against
infection in the GI tract.
 Human milk contains iron-binding protein that exerts a
bacteriostatic effect on E. coli
 Human milk also contains macrophages and lymphocytes.
 There is evidence that early enteral feedings with human milk
are beneficial in establishing a natural barrier to infection in
ELBW and VLBW infants
 There is evidence that early enteral feedings with human milk
are beneficial in establishing a natural barrier to infection in
ELBW and VLBW infants
intravenous (IV) infusion
 Monitoring an intravenous (IV) infusion and administering
antibiotics are important nursing responsibilities.
 It is important to administer the prescribed dose of antibiotic
within 1 hour after it is prepared to avoid loss of drug
stability.
 If the IV fluid that the infant is receiving contains electrolytes,
vitamins, or other medications, the nurse should check with
the hospital pharmacy before adding antibiotics.
 The antibiotic (or other medication) may be deactivated or
may form a precipitate when combined with other
medications.
Care must be taken in suctioning secretions from any newborn’s
oropharynx or trachea
 Routine suctioning is not recommended and may further
compromise the infant’s immune status, cause hypoxia, and
increase ICP
 Efforts should also be taken to prevent ventilatorassociated
pneumonia in infants on mechanical ventilation
MATERNAL INFECTIONS
 Some maternal infections, especially during early gestation,
can result in fetal loss or malformations because the fetus’s
ability to handle infectious organisms is limited and the fetal
immunologic system is unable to prevent the dissemination of
infectious organisms to the various tissues.
 Not all prenatal infections produce teratogenic effects.
o the clinical picture of disorders caused by transplacental
transfer of infectious agents is not always well defined.
o Some viral agents can cause remarkably similar
manifestations, and it is common to test for all of them
when a prenatal infection is suspected.
o This is the so-called TORCH complex, an acronym for:
 T—Toxoplasmosis
 O—Other (e.g., HBV, parvovirus, HIV, West Nile)
 R—Rubella
 C—CMV infection
 H—Herpes simplex
A. DRUG-EXPOSED INFANTS
 Maternal habits hazardous to the fetus and neonate include
drug addiction, smoking, and alcohol abuse.
 Narcotics, which have a low molecular weight, readily cross
the placental membrane and enter the fetal system.
 Illicit substances may also be transmitted to the newborn
through breast milk.
 When the mother is a habitual user of opiates, especially
OxyContin, heroin, or methadone, the unborn child may also
become chemically dependent or passively addicted to the
drug,
o places such infants at risk during the perinatal and
early neonatal periods
 Neonatal abstinence syndrome (NAS) is the term used to
describe the set of behaviors exhibited by infants exposed to
narcotics in utero
INFECTIONS ACQUIRED FROM THE MOTHER BEFORE,
DURING, OR AFTER BIRTH (FETAL OR NEW BORN
EFFECT)
Human Immunodeficiency Virus
 No significant difference between infected and uninfected infants
at birth in some instances
 Embryopathy reported by some observers:
• Depressed nasal bridge
• Mild upward or downward obliquity of eyes
• Long palpebral fissures with blue sclerae
• Patulous lips
• Ocular hypertelorism
• Prominent upper vermilion border
Chickenpox (Varicella-Zoster Virus [VZV])
 Intrauterine exposure—congenital varicella syndrome: limb
dysplasia, microcephaly, cortical atrophy, chorioretinitis, cataracts,
cutaneous scars, other anomalies, auditory nerve palsy, motor and
cognitive delays
 Severe symptoms (rash, fever) and higher mortality in infant whose
mother develops varicella 5 days before to 2 days after birth
Coxsackievirus (Group B Enterovirus-Nonpolio, Parechovirus)
 Poor feeding, vomiting, diarrhea, fever; cardiac enlargement,
arrhythmias, congestive heart failure; lethargy, seizures,
meningoencephalitis, pneumonitis
 Mimics bacterial sepsis
Cytomegalovirus (CMV)
 Variable manifestation from asymptomatic to severe Microcephaly,
cerebral calcifications, chorioretinitis Jaundice,
hepatosplenomegaly Petechial or purpuric rash
 Neurologic sequelae—seizure disorders, sensorineural hearing loss,
cognitive impairment
Parvovirus B19 (Erythema Infectiosum)
 Fetal hydrops and death from anemia and heart failure with early
exposure
 Anemia with later exposure
 No teratogenic effects established Ordinarily low risk of adverse
effect to fetus
Gonococcal Disease (Neisseria gonorrhoeae)
 Ophthalmitis
 Neonatal gonococcal arthritis, septicemia, meningitis
Hepatitis B Virus (HBV)
 May be asymptomatic at birth; more than 90% of infants infected
perinatally develop chronic Hep B infection
 Clinical hepatitis, jaundice, changes in liver function; possible
fulminant hepatitis
Listeriosis (Listeria monocytogenes)
 Maternal infection associated with spontaneous abortion, preterm
birth, and fetal death
 Preterm birth, sepsis, and pneumonia seen in early-onset disease;
late-onset disease usually manifests as meningitis
Rubella, Congenital (Rubella Virus)
 Congenital rubella syndrome
 Eyes-—retinopathy, cataracts (unilateral or bilateral),
microphthalmia, retinitis, glaucoma
 CNS signs—microcephaly, seizures, severe cognitive impairment
 Congenital heart defects—patent ductus arteriosus
 Auditory defects—sensorineural hearing loss
 Dermal erythropoiesis—blueberry muffin lesions
 IUGR—hyperbilirubinemia, meningitis, thrombocytopenia,
hepatomegaly
Syphilis, Congenital (Treponema pallidum)
 Stillbirth, prematurity, hydrops fetalis
 May be asymptomatic at birth and in first few weeks of life or may
have multisystem manifestations: hepatosplenomegaly,
lymphadenopathy, hemolytic anemia, pneumonia, and
thrombocytopenia
 Copper-colored maculopapular cutaneous lesions (usually after
first few weeks of life), mucous membrane patches, hair loss, nail
exfoliation, snuffles (syphilitic rhinitis), profound anemia, poor
feeding, pseudoparalysis of one or more limbs, dysmorphic teeth
(older child)
Herpes Simplex Virus (HSV)
 Neonatal herpes manifests in one of three ways: (1) with SEM
involvement; (2) as localized CNS disease; or (3) as disseminated
disease involving multiple organs. In skin and eye disease rash
appears as vesicles or pustules on erythematous base.
 Clusters of lesions are common. Lesions ulcerate and crust over
rapidly
 Ophthalmologic clinical findings include chorioretinitis and
microphthalmia; neurologic involvement such as microcephaly and
encephalomalacia may also develop. Disseminated infections may
involve virtually every organ system; but liver, adrenal glands, and
lungs are most commonly affected.
 In HSV meningitis, infants develop multiple lesions of cortical
hemorrhagic necrosis. It can occur alone or with SEM lesions.
Presenting symptoms, which may occur in second-to-fourth weeks
of life, include lethargy, poor feeding, irritability, and local or
generalized seizures. Neonatal HSV has high mortality rate.
Group B Streptococcus
 Early-onset-infection (first 24 hours of life)—pneumonia,
respiratory distress, shock, apnea, and meningitis
 Late-onset—(3 to 4 days of age)—bacteremia or meningitis;
may occur later in LBW infants
 The adverse effects of exposure of a fetus to drugs are varied
 Approximately 55% to 94% of infants born to narcotic-addicted
mothers show signs of withdrawal
 Because of irregular and varying degrees of drug use, quality of
drug, and mixed-drug usage by the mother, some infants display
mild or variable manifestations
 Most manifestations are the vague, nonspecific signs characteristic
of all infants in general; therefore it is important to differentiate
between drug withdrawal and other disorders before specific
therapy is instituted. Other conditions (e.g., hypocalcemia,
hypoglycemia, sepsis) often coexist with the drug withdrawal.
 Additional signs seen in drug-exposed newborns include loose
stools; tachycardia; fever; projectile vomiting; crying; nasal
stuffiness; and generalized perspiration, which is unusual in
newborns.
 Newborn urine, hair, or meconium sampling may be required to
identify drug exposure and implement appropriate early
interventional therapies aimed at minimizing the consequences of
intrauterine drug exposure.
 The prognosis for drug-exposed infants depends on the type and
amount of drug(s) taken by the mother and the stage(s) of fetal
development in which the drug was taken
 Often drug-exposed infants exhibit poor brain and body growth at
birth
 early identification of substance abuse in the pregnant woman so
CARE MANAGEMENT
treatment can be initiated and side effects minimized
 If the mother has had good prenatal care, the practitioner is aware
of the problem and may have instituted therapy before birth
o However, a number of mothers deliver their infants
without the benefit of adequate care, and the condition is
unknown to health care personnel at the time of birth.
 The degree of withdrawal is closely related to the amount of drug
the mother has habitually taken, the length of time she has been
taking the drug, and her drug level at the time of birth.
 The most severe symptoms are observed in the infants of mothers
who have taken large amounts of drugs over a long period. In
addition, the nearer to the time of birth that the mother takes the
drug, the longer it takes the child to develop withdrawal, and the
more severe the manifestations.
 The infant may not exhibit withdrawal symptoms until 7 to 10 days
after birth, by which time most newborns have been discharged
from the birth center, and caregivers are less likely to recognize
signs of irritability and poor feeding as withdrawal, thus
predisposing the newborn to abuse or neglect and growth failure
(failure to thrive).
 The infant may be at further risk for subsequent abuse or neglect
because of home conditions that preclude adequate newborn care
and follow-up.
 nursing care is directed toward treating the presenting signs,
o decreasing stimuli that may precipitate hyperactivity and
irritability (e.g., dimming the lights, decreasing noise
levels),
o providing adequate nutrition and
o hydration, and
o promoting the mother-infant relationship.
 Breastfeeding is encouraged
 The Neonatal Abstinence Scoring System or Finnegan tool was
developed to monitor infants in an objective manner and evaluate
their response to clinical and pharmacologic interventions
 Loose stools, poor intake, and regurgitation after feeding
predispose these infants to malnutrition, dehydration, skin
breakdown, and electrolyte imbalance.
 A valuable aid to anticipating problems in the newborn is
recognizing substance abuse in the mother
ALCOHOL EXPOSURE
 Alcohol ingestion during pregnancy is associated with both
shortand long-term effects on the fetus and newborn.
 infants born to heavy drinkers have twice the risk of congenital
abnormalities than those born to moderate drinkers
 Alcohol withdrawal can occur in neonates, particularly when
maternal ingestion occurs near the time of birth.
 SIGNS AND SYMPTOMS
o include jitteriness,
o increased tone and r
o eflex responses, and
o irritability.
o Seizures are also common
 Infants who do not display the signs of FAS (FETAL ALCOHOL
SYNDROME) but are born to mothers who are also heavy
alcohol drinkers have significantly more tremors, hypertonia,
restlessness, excessive mouthing movements, crying, and
inconsolability than infants of substance-abusive mothers who do
not consume alcohol during pregnancy
COCAINE EXPOSURE
 Cocaine is a CNS stimulant and peripheral sympathomimetic.
Legally it is classified as a narcotic, but it is not an opioid.
 The effects on fetuses are secondary to maternal effects,which
include
o increased blood pressure (BP),
o decreased uterine blood flow, and
o increased vascular resistance
o Consequently the fetus experiences decreased blood
flow and oxygenation because of placental and fetal
vasoconstriction
 Infants may appear normal or may show neurologic problems
at birth that may continue during the neonatal period
 Either of two types of behavior may emerge as a result of the
effects of cocaine on fetal development:
o neurobehavioral depression or
o excitability
 sequelae of prenatal cocaine exposure include preterm birth,
o a smaller head circumference,
o decreased birth length, and
o decreased weight.
o Head growth may be one of the best predictors of
longterm development
 Nursing care of cocaine-exposed infants is the same as that for
CARE MANAGEMENT
other drug-exposed infants
 Because they have increased flexor tone, these infants respond
to swaddling
 Positioning, infant massage, and limited tactile stimulation
have been shown to be effective interventions.
 Significant amounts of cocaine have been found in breast milk
o therefore mothers should be cautioned regarding this
hazard to their infants
 Referral to early intervention programs, including
o child health care,
o parental drug treatment,
o individualized developmental care, and
o parenting education, is essential in promoting optimum
outcome for these children.
 Because they often live in impoverished
environments, they are at high risk for cognitive
delays, lack of child health care, and inadequate
nutrition and benefit from early intervention
programs.
Marijuana Exposure
 Marijuana crosses the placenta; however, specific effects on the
fetus have been difficult to determine.
 Some studies have reported an association between the chronic use
of marijuana and a decrease in fetal growth and infant birth weight
and length
o however, this finding is confounded by cigarette smoking
 Compounding the issue of the effects of marijuana, especially
among women ages 18 to 30 years is multidrug use, which
combines the harmful effects of
o marijuana,
o tobacco,
o alcohol,
o opiates, and
o cocaine.
 Long-term follow-up studies on exposed infants are needed
HEMOLYTIC DISORDER
 Hyperbilirubinemia in the first 24 hours of life is most often
the result of hemolytic disease of the newborn (HDN)
(erythroblastosis fetalis), an abnormally rapid rate of red
blood cell (RBC) destruction
 Anemia caused by this destruction stimulates the production
of RBCs, which in turn provides increasing numbers of cells
for hemolysis. Major causes of increased erythrocyte
destruction are isoimmunization (primarily Rh) and ABO
incompatibility
Blood Incompatibility
 The membranes of human blood cells contain a variety of antigens,
also known as agglutinogens, substances capable of producing an
immune response if recognized by the body as foreign.
 The reciprocal relationship between antigens on RBCs and
antibodies in the plasma causes agglutination (clumping).
o In other words antibodies in the plasma of one blood group
(except the AB group, which contains no antibodies) produce
agglutination when mixed with antigens of a different blood
group.
o In the ABO blood group system, the antibodies occur naturally.
In the Rh system the person must be exposed to the Rh antigen
before significant antibody formation takes place and causes a
sensitivity response known as isoimmunization
Rh Incompatibility (Isoimmunization)
 The Rh blood group consists of several antigens (with D being
the most prevalent).
 The presence or absence of the naturally occurring Rh factor
determines the blood type
 Ordinarily no problems are anticipated when the Rh blood
types are the same in both the mother and the fetus or when
the mother is Rh positive and the infant is Rh negative
 Difficulty may arise when:
o mother is Rh negative and the infant is Rh positive
 more commonly fetal RBCs enter into the maternal circulation
at the time of birth. The mother’s natural defense mechanism
responds to these alien cells by producing anti-Rh antibodies.
 Under normal circumstances this process of isoimmunization
has no effect during the first pregnancy with an Rh-positive
fetus because the initial sensitization to Rh antigens rarely
occurs before the onset of labor
o However, with the increased risk of fetal blood
being transferred to the maternal circulation during
placental separation, maternal antibody production
is stimulated.
 During a subsequent pregnancy with an Rh-positive fetus,
o these previously formed maternal antibodies to Rh-
positive blood cells may enter the fetal circulation,
where they attack and destroy fetal erythrocytes
 risk for subsequent isoimmunisation
o Multiple gestations,
o abruptio placentae,
o placenta previa,
o manual removal of the placenta, and
o cesarean birth

Because the condition begins in utero
- the fetus attempts to compensate for the progressive
hemolysis and anemia by accelerating the rate of
erythropoiesis.
- As a result, immature RBCs (erythroblasts) appear in the fetal
circulation; thus the term erythroblastosis fetalis
ABO Incompatibility
 Hemolytic disease can also occur when the major blood group
antigens of the fetus are different from those of the mother.
 The major blood groups are A, B, AB, and O
 The most common blood group incompatibility in the neonate
is between a mother with O blood group and an infant with A
or B blood group
 Naturally occurring anti-A or anti-B antibodies already
present in the maternal circulation cross the placenta and
attack the fetal RBCs, causing hemolysis
 Usually the hemolytic reaction is less severe than in Rh
incompatibility; however, rare cases of hydrops have been
reported
 Unlike the Rh reaction, ABO incompatibility may occur in the
first pregnancy.
 The risk of significant hemolysis in subsequent pregnancies is
higher when the first pregnancy is complicated by ABO
incompatibility
 Jaundice may appear shortly after birth (during the first 24
hours) in newborns affected by HDN
 serum levels of unconjugated bilirubin rise rapidly
 Anemia results from the hemolysis of large numbers of
erythrocytes, and hyperbilirubinemia and jaundice result from
the inability of the liver to conjugate and excrete the excess
bilirubin
 Most newborns with HDN are not jaundiced at birth.
However, hepatosplenomegaly and varying degrees of
hydrops may be evident
 If the infant is severely affected, signs of anemia (notably
marked pallor) and hypovolemic shock are apparent.
 Hypoglycemia may occur as a result of pancreatic cell
hyperplasia
 Early identification and diagnosis of Rh(D) sensitization are
important in the management and prevention of fetal
complications.
o A maternal antibody titer (indirect Coombs’ test)
should be drawn at the first prenatal visit
o Genetic testing allows early identification of
paternal zygosity at the Rh(D) gene locus, thus
allowing earlier detection of the potential for
isoimmunization and avoiding further maternal or
fetal testing
 Amniocentesis can be used to test the fetal blood type of a
woman whose antibody screen result is positive; the use of
PCR may determine the fetal blood type and presence of
maternal antibodies
 The fetal hemoglobin and hematocrit can also be measured.
 Chorionic villus sampling has drawbacks that preclude its
use, including possible spontaneous abortion of the fetus and
fetomaternal hemorrhage, which would essentially make the
situation worse.
 Ultrasonography is considered an important adjunct in the
detection of isoimmunisation.
o alterations in the placenta, umbilical cord, and
amniotic fluid volume and the presence of fetal
hydrops can be detected with high-resolution
ultrasonography and allow early treatment before
the development of erythroblastosis
 Doppler ultrasonography of fetal middle cerebral artery
peak velocity has been used to detect and measure fetal
hemoglobin and subsequently fetal anemia
 Erythroblastosisfetalis caused by Rh incompatibility can also be
monitored by evaluating rising anti-Rh antibody titers in the
maternal circulation or testing the optical density of amniotic fluid
(delta OD450 test) because bilirubin discolors the fluid.
 The disease in the newborn is suspected on the basis of the timing
and appearance of jaundice and can be confirmed postnatally by
detecting antibodies attached to the circulating erythrocytes of
affected infants (direct Coombs’ test or direct antiglobulin test).
 The Coombs’ test may be performed on umbilical cord blood
samples from infants born to Rh-negative mothers if there is a
history of incompatibility or further investigation is warranted.
 The primary aim of therapeutic management ofisoimmunization is
prevention.
PREVENTION
 The administration of RhIg, a human gamma globulin concentrate
of anti-D, to all unsensitized Rh-negative mothers after birth or
abortion of an Rh-positive infant or fetus prevents the development
of maternal sensitization to the Rh factor.
 The injected anti-Rh antibodies are thought to destroy (by
subsequent phagocytosis and agglutination) fetal RBCs passing
into the maternal circulation before they can be recognized by the
mother’s immune system.
o Because the immune response is blocked, anti-D
antibodies and memory cells (which produce the primary
and secondary immune responses, respectively) are not
formed (Bagwell, 2007; Blackburn, 2011).
o The inhibition of memory cell formation is especially
important because memory cells provide long-term
immunity by initiating a rapid immune response after the
antigen is reintroduced (McCance and Huether, 2010).
 To be effective, RhIg (e.g., RhoGAM) must be administered to
unsensitized mothers within 72 hours (but possibly as long as 3 to
4 weeks) after the first birth or abortion and repeated after
subsequent pregnancies or losses.
 The administration of RhIG at 26 to 28 weeks of gestation further
reduces the risk of Rh isoimmunization. RhIg is not effective
against existing Rh-positive antibodies in the maternal circulation.
 IVIG administered to the neonate is believed to attack the maternal
cells that destroy neonatal RBCs, slowing the progression of
bilirubin production (Mundy, 2005).
o This therapy, often used in conjunction with
phototherapy, may decrease the necessity for exchange
transfusion.
o Maternal administration of high-dose IVIG, alone or in
combination with plasmapheresis, decreases the fetal
effects of Rh(D) isoimmunization (Moise, 2008b;
Urbaniak, 2008).
INTRAUTERINE TRANSFUSION
 Infants of mothers already sensitized may be treated by intrauterine
transfusion, which consists of infusing blood into the umbilical
vein of the fetus.
 The need for therapy is based on the antenatal diagnosis of
fetalanemia by serial Doppler assessments of peak systolic velocity
of the middle cerebral artery (Moise and Argot, 2012).
o With the advance of ultrasound technology, fetal transfusion
may be accomplished directly via the umbilical vein, infusing
type O Rh-negative packed RBCs to raise the fetalhematocrit
to 40% to 50%; fetal movement and transfusion risks are
minimized by administering vecuronium bromide for
temporary fetal paralysis.
 The frequency of intrauterine transfusions may vary according to
institution and fetalhydropic status, but
o one recommendation is for intervals of 10 days, 2 weeks, and
then 3 weeks for subsequent procedures until the fetus reaches
pulmonary maturity at approximately 37 to 38 weeks of
gestation
 Intraperitoneal blood transfusions are used less commonly for
isoimmunization because of higher associated fetal risks;
o however, they may be used when intravascular access is
impossible.
EXCHANGE TRANSFUSION
 Exchange transfusion, in which the infant’s blood is removed in
small amounts (usually 5 to 10 mL at a time) and replaced with
compatible blood (e.g., Rh-negative blood), is a standard mode of
therapy for treatment of severe hyperbilirubinemia and is the
treatment of choice for hyperbilirubinemia and hydrops caused by
Rh incompatibility.
 Exchange transfusion removes the sensitized erythrocytes, lowers
the serum bilirubin level to prevent bilirubin encephalopathy,
corrects the anemia, and prevents cardiac failure.
 Indications for exchange transfusion in full-term infants may
include a rapidly increasing serum bilirubin level and hemolysis
despite intensive phototherapy.
 An infant born with hydropsfetalis or signs of cardiac failure is a
candidate for immediate exchange transfusion with fresh whole
blood.
o For exchange transfusion fresh whole blood is typed and
crossmatched to the mother’s serum.
 The amount of donor blood used is usually double the blood
volume of the infant, which is approximately 85 mL/kg body
weight but is limited to no more than 500 mL.
 The two-volume exchange transfusion replaces approximately 85%
of the neonate’s blood. An exchange transfusion is a sterile surgical
procedure.
o A catheter is inserted into the umbilical vein and threaded into
the inferior vena cava.
 Depending on the infant’s weight, 5 to 10 mL of blood is
withdrawn within 15 to 20 seconds, and the same volume of donor
blood is infused over 60 to 90 seconds.
-CARE MANAGEMENT-
 The initial nursing responsibility is recognizing jaundice in the
newborn at risk.
 The possibility of hemolytic disease can be anticipated from the
prenatal and perinatal history.
o Prenatal evidence of incompatibility and a positive
Coombs’ test result are cause for increased vigilance for
early signs of jaundice in an infant.
 Data indicate that the hour-specific bilirubin nomogramcan be used
in infants born at 35 weeks or more with ABO incompatibility and
a positive Coombs’ test result to follow the infant’s serum bilirubin
to determine the need for additional follow-up after hospital
discharge.
o If an exchange transfusion is required, the nurse prepares
the infant and the family and assists the practitioner with
the procedure.
o The infant receives nothing by mouth (NPO) during the
procedure; therefore a peripheral infusion of dextrose and
electrolytes is established.
o The nurse documents the blood volume exchanged,
including the amount of blood withdrawn and infused,
the time of each procedure, and the cumulative record of
the total volume exchanged.
o Vital signs monitored electronically are evaluated
frequently and correlated with the removal and infusion
of blood.
o If signs of cardiac or respiratory problems occur, the
procedure is stopped temporarily and resumed after the
infant’s cardiorespiratory function stabilizes.
o The nurse also observes for signs of blood transfusion
reaction and maintains the infant’s blood glucose levels
and fluid balance.
o Throughout the procedure attention must be given to the
infant’s thermoregulation.
o Hypothermia increases oxygen and glucose consumption,
causing metabolic acidosis.
 Not only do these consequences hinder the infant’s overall physical
ability to withstand the long procedure, but they also inhibit the
binding capacity of albumin and bilirubin and the hepatic
enzymatic reactions, thus increasing the risk of kernicterus.
o Conversely hyperthermia damages the donor
erythrocytes, elevating the free potassium content and
predisposing the infant to cardiac arrest.
o The exchange transfusion is performed with the infant in
a radiant warmer.
o However, he or she is usually covered with sterile drapes
that may prevent the radiant heat from sufficiently
warming the skin.
o The blood may also be warmed (using specially designed
blood warming devices only) before infusion.
 After the procedure is completed the nurse inspects the umbilical
site for evidence of bleeding.
 The catheter may remain in place in case repeated exchanges are
required.
INFANTS OF DIABETIC MOTHERS
Before insulin therapy few women with diabetes were able to
conceive
o for those who did, the mortality rate for both the mother
and infant was high.
The morbidity and mortality of infants of diabetic mothers (IDMs)
have been reduced significantly as a result of effective control of
maternal diabetes and an increased understanding of fetal disorders
infants born to women with gestational diabetes mellitus (DM) are
at risk for the same complications as IDM’s
The severity of maternal diabetes affects infant survival.
o It is determined by the duration of the disease before
pregnancy
o age of onset; extent of vascular complications;
o and abnormalities of the current pregnancy such as
 pyelonephritis,
 diabetic ketoacidosis,
 pregnancy-induced hypertension, and
 noncompliance.
The single most important factor influencing fetal well-being is the
euglycemic status of the mother.
o It has been found that reasonable metabolic control that
begins before conception and continues during the first
weeks of pregnancy can prevent malformation in an IDM
Hypoglycemia may appear a short time after birth and in IDMs is
associated with increased insulin activity in the blood
The serum glucose level that corresponds to clinical hypoglycemia
has not been well defined.
o Because some infants experience metabolic complications
at higher levels than previously thought, some researchers
recommend that serum glucose levels be maintained above
45 mg/ dL (2.5 mmol/L) in infants with abnormal clinical
symptoms and as high as 50 or 60 mg/dL in other infants
Hypoglycemia in IDMs is related to hypertrophy and hyperplasia
of the pancreatic islet cells and thus is a transient state of
hyperinsulinism
High maternal blood glucose levels during fetal life provide a
continual stimulus to the fetal islet cells for insulin production
(glucose easily passes the placental barrier from maternal to fetal
side; however, insulin does not cross the placental barrier).
Historically, maternal hyperglycemia was believed to contribute to
fetal macrosomia.
o However, Hay (2012) suggests that maternal
hyperlipidemia and increased lipid transfer to the fetus are
responsible for the excessive weight gain and fat deposition
seen in such infants
IDMs are more likely to have disproportionately large abdominal
circumferences and shoulders, leading to an increased risk of
shoulder dystocia and birth injury
Infants of mothers with advanced diabetes may be small for
gestational age, have IUGR, or be the appropriate size for
gestational age because of the maternal vascular (placental)
involvement.
There is an increase in congenital anomalies in IDMs in addition to
a high susceptibility to hypoglycemia, hypocalcemia,
hypomagnesemia, polycythemia, hyperbilirubinemia,
cardiomyopathy, and respiratory distress syndrome (RDS)

Central nervous system (CNS) anomalies such as anencephaly,
spina bifida, and holoprosencephaly occur at rates 10 times higher
than in any other population of mothers.
Cardiac anomalies such as ventriculoseptal defects and coarctation
of the aorta are increased fivefold in IDMs, and sacral agenesis and
caudal regression occur almost exclusively in IDMs
Hyperinsulinemia and hyperglycemia in the diabetic mother may be
factors in reducing fetal surfactant synthesis, thus contributing to the
development of RDS. Although large, these infants may be delivered
before term as a result of maternal complications or increased fetal size.
Congenital hyperinsulinism is a condition that causes neonatal
macrosomia, and profound hypoglycemia is often present in the
neonatal period.
o However, this condition is usually not associated with
maternal DM, but appears to have a genetic etiology; the
condition is also associated with syndromes such as
Beckwith-Wiedemann syndrome (Sperling, 2011).
Some IDMs are also at increased risk for deep vein thrombosis,
with renal vein thrombosis and hematuria being the most common
presentation (Hay, 2012).
o Additional problems in IDMs include perinatal iron
deficiency and neurologic impairments (seizures,
lethargy, jitteriness, and changes in tone) (Hay, 2012).
The most important management of IDMs is careful
monitoring of serum glucose levels and observation for
accompanying complications such as RDS and cardiac
anomalies.
The infants are examined for the presence of any anomalies or birth
injuries; and blood studies for determination of glucose, calcium,
hematocrit, and bilirubin are obtained on a regular basis.
o Because the hypertrophied pancreas is so sensitive to
blood glucose concentrations, the administration of oral
glucose may trigger a massive insulin release, resulting
in rebound hypoglycemia.
o Therefore feedings of breast milk or formula begin
within the first hour after birth, provided that the infant’s
cardiorespiratory condition is stable.
o Approximately half of these infants do well and adjust
without complications.
Infants born to mothers with poorly controlled diabetes may
require IV dextrose infusions.
o Treatment with 10% dextrose and water intravenously is
initiated with the goal of maintaining serum blood
glucose levels between 40 and 50 mg/dL (Adamkin and
AAP, Committee on Fetus and Newborn, 2011).
Oral and IV intake may be titrated to maintain adequate blood
glucose levels.
o Frequent blood glucose determinations are needed for the
first 2 to 4 days of life to assess the degree of
hypoglycemia present at any given time.
o Testing blood taken from the heel with calibrated
portable reflectance meters (e.g., glucometers) is a
simple and effective screening evaluation that can then
be confirmed by laboratory examination.
CARE MANAGEMENT
The nursing care of IDMs involves early examination for
congenital anomalies, signs of possible respiratory or cardiac
problems, maintenance of adequate thermoregulation, early
introduction of carbohydrate feedings as appropriate, and
monitoring of serum blood glucose levels.
The latter is of particular importance because many infants with
hypoglycemia may remain asymptomatic.
o Symptomatic IDMs who are unable to feed should be
started on a continuous intravenous infusion of 10%
dextrose at 4 to 6 mg/min/kg unless blood glucose is
below 20 mg/dL.
In such cases a one-time bolus infusion of 10% dextrose (200
mg/kg) should be given over 2 to 4 minutes, followed by a constant
intravenous infusion of 10% dextrose and water as noted
previously
IV glucose infusion requires careful monitoring of the site and the
neonate’s reaction to therapy; high glucose concentrations
(≥12.5%) should be infused via a central line instead of a
peripheral site.
Because macrosomic infants are at risk for problems
associated with a difficult birth, they are monitored for birth
injuries such as brachial plexus injury and palsy, fractured
clavicle, and phrenic nerve palsy.
o Additional monitoring of the infant for problems
associated with this condition (polycythemia,
hypocalcemia, poor feeding, and hyperbilirubinemia) is
also a vital nursing function.
Some evidence indicates that IDMs have an increased risk of
acquiring type 2 diabetes and metabolic syndrome in childhood or
early adulthood (Hay, 2012); therefore nursing care should also
focus on healthy lifestyle and prevention later in life with IDMs
CONGENITAL ANOMALIES
 Congenital defects are reported to occur in 2% to 3% of all
live births
o increases to about 6% by 5 years,
 the incidence of congenital malformations in fetuses that are
aborted is higher than that in infants who are born alive,
 Between 1999 and 2008 rates of cleft lip and cleft palate had
the highest prevalence followed by Down syndrome,
omphalocele or gastroschisis, spina bifida or
myelomeningocele, and anencephaly.
 Prevalence of Down syndrome was highest among mothers 40
to 54 years of age, and rates of omphalocele or gastroschesis
were highest among mothers younger than 20 years of age
 The most common major congenital anomalies that cause
serious problems in the neonate are
o congenital heart disease,
o abdominal wall defects,
o imperforate anus,
o neural tube defects (NTDs),
o cleft lip or palate,
o clubfoot, and
o developmental dysplasia of the hip
 Most congenital anomalies are detected at birth or shortly
thereafter.
 Ways of detecting and preventing some of these anomalies are
being improved continuously, as are some surgical techniques
for the care of the fetus with certain anomalies
Cleft Lip and Cleft Palate
 Clefts of the lip (CL) and palate (CP) are facial malformations
that occur during embryonic development and are the most
common congenital deformities in the United States
 They may appear separately or more often together
 CL results from failure of the maxillary and median nasal
processes to fuse
 CP is a midline fissure of the palate that results from failure of
the two palatal processes to fuse.
 The palate can be divided into the primary and secondary
palates.
The primary palate
  consists of the medial portion of the upper lip and the
portion of the alveolar ridge that contains the central and
lateral incisor
The secondary palate
 consists of the remaining portion of the hard palate and
all of the soft palate.
 CL may vary from a small notch in the upper lip to a complete cleft
extending into the base of the nose, including the lip and the
alveolar ridge
 CL can be unilateral or bilateral
 Deformed dental structures are associated with CL
 Isolated CP occurs in the midline of the secondary palate and may
also vary from a bifid uvula (the mildest form of CP) to a complete
cleft extending from the soft to the hard palate.
Etiology
 Cleft deformities may be an isolated anomaly, or they may
occur with a recognized syndrome
 CL/P and CP are distinct from isolated CP
 Clefts of the secondary palate alone are more likely to be
associated with syndromes than are isolated CL or CL/P
 Most cases of CL/P have multifactorial inheritance,
o generally caused by a combination of genetic and
environmental factors.
 Exposure to teratogens such as alcohol, cigarette smoking,
anticonvulsants, steroids, and retinoids are associated with
higher rates of oral clefting. Folate deficiency is also a risk
factor for clefting.
Therapeutic Management
 Treatment of the child with CL/P involves the cooperative
efforts of a cleft/craniofacial multidisciplinary health care
team, including
o pediatrics,
o plastic surgery,
o orthodontics,
o otolaryngology,
o speech/ language pathology,
o audiology,
o nursing, and social work.
 Management is directed toward closure of the cleft(s),
prevention of complications, and facilitation of normal growth
and development in the child.
Surgical Correction of Cleft Lip
 CL repair typically occurs at most centers between 2 and 3
months of age
 Common procedures for repair of CL include
o ennison-Randall triangular flap (Z-plasty),
o the Fisher technique, and t
o he Millard rotational advancement technique.
 Nasoalveolar molding (NAM) may also be used to bring the cleft
segments closer together before definitive CL repair, reducing
the need for CL revision
Surgical Correction of Cleft Palate.
 CP repair typically occurs between 6 and 12 months.
 There is concern that early CP repair interferes with skeletal
growth of the midface, but postponing palate closure
beyond the child’s first words may result in increased
speech disorders
 Common techniques to repair CP include
o Veau-Wardill-Kilner V-Y pushback procedure
and
o the Furlow double-opposing Z-plasty.
 Approximately 20% to 30% of children with repaired CP
need a secondary surgery to improve velopharyngeal
closure for speech.
 A small number of these children will have velopharyngeal
dysfunction with hypernasality and nasal air emission.
 Secondary procedures may include
o palatal lengthening,
o pharyngeal flap,
o sphincter pharyngoplasty, or
o posterior pharyngeal wall augmentation
o to improve velopharyngeal closure
 If the child is not a candidate for surgical revision to
improve velopharyngeal function, prosthetic management
may be considered.
CARE MANAGEMENT
Feeding.
 Feeding the infant with a cleft presents a challenge to nurses and
parents
 Growth failure in infants with CL/P or CP has been attributed to
preoperative feeding difficulties
 After surgical repair most infants who have isolated CL, CP, or
CL/P with no associated syndromes gain weight or achieve
adequate weight and height for age
 An infant with an isolated CL typically has no difficulty
breastfeeding because the breast tissue is able to conform to the
cleft
 Cheek support (squeezing the cheeks together to decrease the width
of the cleft) may be useful in improving lip seal during feeding
 Infants with CP and CL/P are often unable to feed using
conventional methods before surgical management
 CP reduces the infant’s ability to suck, which interferes with
breastfeeding and traditional bottle-feeding.
 Modifications to positioning, bottle selection, and feeder
supportive techniques can help infants with CP feed efficiently
 Begin by positioning an infant with CP in an upright position with
the head supported by the caregiver’s hand or cradled in the arm
 this position allows gravity to assist with the flow of the
liquid so it is swallowed instead of resulting in a loss of
liquid through the nose
 Several types of bottles work well with infants unable to generate
adequate suction, including the Special Needs Feeder (formerly
Haberman), the Pigeon bottle, and the Mead-Johnson Cleft Palate
Nurser.
 Infants with clefts tend to swallow excessive air during feedings;
thus it is important to pause during feedings and burp the infant
 Regardless of the feeding method used, the mother should begin
feeding the infant as soon as possible
 preferably after the initial nursery feeding
Preoperative Care
In preparation for surgical repair, parents may be taught to use
alternative feeding systems (e.g., syringes) several days before surgery.
Postoperative Care
 major efforts in the postoperative period are directed toward
protecting the operative site
 For CL parents may be advised to apply petroleum jelly to the
operative site for several days after surgery
 For CL, CP, or CL/P, elbow immobilizers may be used to
prevent the infant from rubbing or disturbing the suture line
o They are applied immediately after surgery and may
be used for 7 to 10 days.
 using a syringe for feeding for 7 to 10 days after CL or CP
repair
 Adequate analgesia is required to relieve postoperative pain
and prevent restlessness
 Feeding is resumed when tolerated
 Avoid the use of suction or other objects in the mouth such as
tongue depressors, thermometers, pacifiers, spoons, and
straws
 The older infant or child may be discharged on a blenderized
or soft diet, and parents are instructed to continue the diet
until the surgeon directs them otherwise
 Parents are cautioned against allowing the child to eat hard
items (e.g., toast, hard cookies, and potato chips) that can
damage the repaired palate
Esophageal Atresia and Tracheoesophageal Fistula
 Congenital EA and tracheoesophageal fistula (TEF) are rare
malformations that represent a failure of the esophagus to develop
as a continuous passage and a failure of the trachea and esophagus
to separate into distinct structures.
 These defects may occur as separate entities or in combination
 without early diagnosis and treatment they pose a serious threat to
the infant’s well-being
Etiology
 EA with or without an associated TEF is the most common
esophageal malformation
 occurring in approximately 90% of the one in 4000 affected
neonates
 There appears to be an equal sex incidence
 birth weight of most affected infants is significantly lower
than average
  A history of maternal polyhydramnios is present in
approximately 50% of infants with the defect
 Approximately 50% of the cases of EA/TEF are a component
of VATER or VACTERL association, acronyms used to
describe associated anomalies
o VATER for vertebral defects, imperforate anus,
tracheoesophageal fistula, and radial and renal
dysplasia; and
o VACTERL for vertebral, anal, cardiac, tracheal,
esophageal, renal, and limb
 Other associated conditions include
o DiGeorge syndrome,
o Down syndrome,
o Pierre-Robin sequence,
o Fanconi syndrome, and
o CHARGE syndrome (coloboma,
o heart defects, choanal atresia,
o developmental restriction,
o genital hypoplasia, and ear deformities
CLINICAL MANIFESTATIONS OF TRACHEOESOPHAGEAL
FISTULA
 Excessive salivation and drooling
 Three C’s of tracheoesophageal fistula:
• Coughing
• Choking
• Cyanosis
 Apnea
 Increased respiratory distress during and after feeding
 Abdominal distention
Diagnostic Evaluation
 The disorder is suspected on the basis of clinical
manifestations
 EA should also be suspected in cases of maternal
polyhydramnios
 Although the diagnosis is established on the basis of clinical
signs and symptoms, the exact type of anomaly is determined
by radiographic studies.
o A radiopaque catheter is inserted into the
hypopharynx and advanced until it encounters an
obstruction
 Chest radiographs are taken to ascertain esophageal patency
or the presence and level of a blind pouch
Therapeutic Management
 The treatment of patients with EA and TEF includes maintenance
of a patent airway, prevention of pneumonia, gastric or blind pouch
decompression, supportive therapy, and surgical repair of the
anomaly
 When EA with a TEF is suspected, the infant is immediately
deprived of oral intake,
 IV fluids are initiated
 the infant is positioned prone or semi-upright to facilitate drainage
of secretions and decrease the likelihood of aspiration
 Accumulated secretions are suctioned frequently from the mouth
and pharynx.
 The infant’s head is kept upright to facilitate removal of fluid
collected in the pouch and prevent aspiration of gastric content
 Most malformations can be corrected surgically in one operation or
in two or more staged procedures.
 The surgery consists of a thoracotomy with division and ligation of
the TEF and an end-to-end or end-to-side anastomosis of the
esophagus
CARE MANAGEMENT
ursing responsibility for detection of this serious malformation
begins immediately after birth
 the major concern is the establishment of a patent airway and
prevention of further respiratory compromise
 Cyanosis is usually a result of laryngeal spasm caused by overflow
of saliva into the larynx from the proximal esophageal pouch or
aspiration
Preoperative Care
 The nurse carefully suctions the mouth and nasopharynx and places
the infant in an optimum position to facilitate drainage and avoid
aspiration.
 Position infant in supine position with the head elevated on an
inclined plane of at least 30 degrees.
o This positioning minimizes the reflux of gastric
secretions at the distal esophagus into the trachea and
bronchi, especially when intraabdominal pressure is
elevated
 It is imperative to immediately remove any secretions that can be
aspirated.
o Until surgery the blind pouch is kept empty by
intermittent or continuous suction through an indwelling
double-lumen or Replogle catheter passed orally or
nasally to the end of the pouch.
 Nursing interventions include respiratory assessment, airway
management, thermoregulation, fluid and electrolyte management,
and PN support
 Often the infant must be transferred to a hospital with a specialized
care unit and pediatric surgical team.
 The nurse advises the parents of the infant’s condition and provides
them with necessary support and information
Postoperative Care
 Postoperative care for these infants is the same as for any
high-risk newborn.
 The infant is returned to a radiant warmer or isolett
 If a thoracotomy is performed and a chest tube is inserted,
attention to the appropriate function of the closed drainage
system is imperative.
 Pain management in the postoperative period is important
even if only a thoracoscopic approach is used.
 In the first 24 to 36 hours the nurse should provide pain
management for the neonate just as for an adult undergoing a
similar surgical procedure
 Tracheal suction should only be done using a premeasured
catheter and with extreme caution to avoid injury to the suture
line.
 If tolerated, gastrostomy feedings may be initiated and
continued until the esophageal anastomosis is healed.
 The nurse must observe the initial attempt at oral feeding
carefully to make certain the infant is able to swallow without
choking
Omphalocele
• Protrusion of intraabdominal viscera into base of umbilical
cord; sac covered with peritoneum without skin
Symptoms
• Usually obvious on inspection; however, small omphalocele
may appear to be a hematoma in umbilical cord Observe for
associated malformations
Care management
Therapeutic:
• Surgical repair of defect
Nursing:
Preoperative:
• Protect defect from trauma or drying.
• Keep sac or viscera moist with saline-soaked dressings.
• Maintain thermoregulation.
• Carry out routine care of IV fluids and line.
• Administer prophylactic antibiotics as prescribed.
 • Provide nasogastric suction for gastric decompression.
N • Keep patient NPO. Assess for associated birth defects such as
CL or CP.
Postoperative:
• Monitor vital signs and BP.
• Assess for and manage pain Bowel decompression–NG tube IV
fluid intake Monitor return of bowel function
GASTROSCHISIS
• Protrusion of intraabdominal contents through defect in abdominal
wall lateral to umbilical ring; no peritoneal sac covering the
exposed bowel
Symptoms
• Defect obvious at delivery if not detected prenatally by These blood flow patterns are
ultrasonography increased pulmonary blood flow;
decreased pulmonary blood flow;
obstruction to blood flow out of the heart;
mixed blood flow, in which saturated and desaturated blood
mix within the heart or great arteries.
 Using this classification system, the clinical presentation and
management of the most common defects are outlined. The
outcomes of surgical treatment for patients with moderate to severe
disease are variable. Patient risk factors for increased morbidity
 and mortality include prematurity or low birth weight, a genetic
syndrome, multiple cardiac defects, a noncardiac congenital
anomaly, and age at time of surgery (neonates are a higher risk
group).
DEFECTS WITH INCREASED PULMONARY BLOOD FLOW

Ventricular Septal Defect

Care management
Description—Abnormal opening between the right and left ventricles.
Therapeutic:
May be classified according to location: membranous (accounting for
• Surgical repair of defect. For large lesions provide gradual
80%); or muscular. May vary in size from a small pinhole to absence of
reduction of abdominal contents via Siloh pouch before
the septum, which results in a common ventricle. VSDs are frequently
surgical closure.
associated with other defects, such as pulmonary stenosis, transposition
Nursing:
of the great vessels, PDA, atrial defects, and COA.
Preoperative
Clinical manifestations —HF is common. There is a characteristic loud
• Keep sac covered with a bowel bag to prevent trauma, drying of
holosystolic murmur heard best at the left sternal border. Patients are at
viscera.
risk for BE and pulmonary vascular obstructive disease.
• NG decompression.
Surgical Treatment
• Maintain thermoregulation.
Palliative —Pulmonary artery banding (placement of a band around the
• Administer IV fluids.
main pulmonary artery to decrease pulmonary blood flow) may be done
• Administer antibiotics.
in infants with multiple muscular VSDs or complex anatomy.
• Observe exposed bowel for signs of necrosis or constriction at
Improvements in surgical techniques and postoperative care make
exit site.
complete
Postoperative:
• repair in infancy the preferred approach.
• Monitor vital signs and BP.
Complete repair (procedure of choice) —Small defects are repaired
• Bowel decompression with NG tube.
with sutures. Large defects usually require that a knitted Dacron patch
• Administer IV fluids.
be sewn over the opening. CPB is used for both procedures. The
• Assess for and manage pain.
approach for the repair is generally through the right atrium and the
• Monitor surgical closure site for infection.
tricuspid valve. Postoperative complications include residual VSD and
• Monitor lower extremities for pulses and circulation (in case of
conduction disturbances.
vena cava compression by large bowel in small abdominal
cavity).
• Monitor for return of bowel function and peristalsis.
• In event of Siloh pouch, nursing care should also include
monitoring vital signs, keeping pouch clean, and aseptic
technique with dressing changes (if not done by surgeon).
Monitor lower extremities for circulation (as noted previously).
• Provide emotional support for parents.
• Long-term problems associated with feeding and weight gain for
gastroschisis and large omphalocele.
CONGENITAL CARDIAC DEFECTS
CARDIOVASCULAR DYSFUNCTION (see chapter 42 pg. 1318)
Cardiovascular disorders in children are divided into two major
groups—congenital heart disease and acquired heart disorders.
Congenital heart disease (CHD)
includes primarily anatomic abnormalities present at birth that
result in abnormal cardiac function. The clinical consequences
of congenital heart defects fall into two broad categories—
heart failure (HF) and hypoxemia.
• Nonsurgical treatment—Device closure during cardiac
Acquired cardiac disorders
catheterization is being performed in some centers under
are disease processes or abnormalities that occur after birth and
investigational protocols. One device has been approved for
can be seen in the normal heart or in the presence of congenital
closure of muscular defects, and another is in clinical trials.
heart defects. They result from various factors, including
Early results are encouraging, with successful defect closure
infection, autoimmune responses, environmental factors, and
and few complications (Rome and Kreutzer, 2004).
familial tendencies.
• Prognosis—Risks depend on the location of the defect, the
CONGENITAL HEART DISEASE
number of defects, and the presence of other associated
includes primarily anatomic abnormalities present at birth that
cardiac defects. Singlemembranous defects are associated
result in abnormal cardiac function.
with low mortality (<2%); multiple muscular defects can carry
CHD is the major cause of death (other than prematurity) in the
a higher risk.
first year of life. Although there are more than 35 well-
recognized cardiac defects,the most common heart anomaly is
ventricular septal defect (VSD).
Congenital diaphragmatic hernia
The exact cause of most congenital cardiac defects is unknown.
• Congenital Diaphragmatic Protrusion of abdominal organs through
MATERNAL RISK FACTORS INCLUDE :
opening in diaphragm, commonly on left side, causing severe
chronic illnesses
respiratory compromise and inability to adequately expand
diabetes
affected lung, which may be hypoplastic.
poorly controlled phenylketonuria
alcohol consumption
exposure to environmental toxins
infections
family history of a cardiac defect in a parent or
first-degree relative (parent or sibling)
Classification of Defects
based on hemodynamic characteristics (blood flow patterns
within the heart)
DIAGNOSTIC AND EVALUATION
• Symptoms: Commonly severe respiratory distress at birth or
within a few hours; tachypnea, cyanosis, dyspnea, a scaphoid
abdomen; absent breath sounds on affected side; impaired cardiac
output; possible symptoms of shock, severe acidosis.
• Milder cases may be seen after birth without severe respiratory
distress.
• Diagnosis: Suspected on basis of symptoms, confirmed by
radiographic study; often diagnosed prenatally as early as 25th
week of gestation
Therapeutic Management:
• Provide prompt recognition, resuscitation, and stabilization.
• Avoid bag and mask ventilation in diagnosed or suspected
• CDH because this fills stomach with air and further compromises
respiratory function.
• Provide supportive treatment of respiratory distress and correction
of pulmonary hypertension and persistence of fetal circulation;
correct acidosis*; endotracheal intubation, GI decompression.
• Additional treatments to reverse pulmonary hypertension may
involve administration of inhaled nitric oxide, use of high-
frequency oscillation, sildenafil, or ECMO.
• Administer prophylactic antibiotics.
• Perform surgical reduction of hernia and repair of defect after
respiratory status is stable (corrected acidosis*; pulmonary
hypertension).
Nursing Management:
• Preoperative:
• Monitor respiratory status; provide supplemental oxygen; assist
with and monitor mechanical ventilation.
• Monitor cardiovascular status; support with inotropes may be
necessary.
• Reduce stimulation—environmental and nursing care activities
(cluster care to prevent constant interruptions).
• Maintain NG suction, oxygen, and IV fluids.
• Administer medications: sedation, muscular paralysis, inotropes,
sildenafil (to reverse pulmonary hypertension).
• Postoperative: • Myelomeningocele (meningomyelocele)—Hernial protrusion
• Carry out routine postoperative care and observation for acutely ill of a saclike cyst containing meninges, spinal fluid, and a
infant. portion of the spinal cord with its nerves.
• Relieve pain and provide comfort. • Anencephaly, the most serious NTD, is a congenital
• Support family because this is a critical illness. malformation in which both cerebral hemispheres are absent.
Spina Bifida (Myelomeningocele) The condition is incompatible with life, and many affected
• Abnormalities that derive from the embryonic neural tube infants are stillborn. no specific treatment is available
(neural tube defects [NTDs]) constitute the largest group of
congenital anomalies that are consistent with multifactorial
inheritance.
• Normally, the spinal cord and cauda equina are encased in a
protective sheath of bone and meninges.
• Failure of neural tube closure produces defects of varying
degrees (Box. 49.4). They may involve the entire length of the
neural tube or may be restricted to a small area.
• In the United States, rates of NTDs have declined from 1.3 per
1000 births in 1970 to 0.3 per 1000 births after the
introduction of mandatory food fortification with folic acid in
1998.

• Myelodysplasia refers broadly to any malformation of the

spinal canal and cord. Midline defects involving failure of
the osseous (bony) spine to close are called spina bifida
(SB), the most common defect of the central nervous system
 (CNS). SB is categorized into two types—SB occulta and SB
cystica.
• Spina bifida occulta refers to a defect that is not visible
externally.
• It occurs most frequently in the lumbosacral area. SB occulta
may not be apparent unless there are associated cutaneous
manifestations or neuromuscular disturbances.
• Spina bifida cystica refers to a visible defect with an external
saclike protrusion. The two major forms of SB cystica:
1. Meningocele, which encases meninges and spinal fluid but no
neural elements.
2. Myelomeningocele (MMC) (or meningomyelocele), which
contains meninges, spinal fluid, and nerves.
• Meningocele is not associated with neurologic
deficit, which occurs in varying, often serious,
degrees in myelomeningocele.
Additional factors predisposing children to an increased risk for NTDs
includes:
• Pre-pregnancy
• maternal obesity
• maternal diabetes mellitus
• Low maternal vitamin B12
• status, maternal hyperthermia
• Use of AEDs in pregnancy.
DIAGNOSTIC AND LABORATORY
• The diagnosis of SB is made on the basis of clinical
manifestations and examination of the meningeal sac.
• Diagnostic measures used to evaluate the brain and spinal
cord includes: MRI, ultrasonography, and CT.
• A neurologic evaluation will determine the extent of
involvement of bowel and bladder function as well as lower
extremity neuromuscular involvement.
• Flaccid paralysis of the lower extremities is a common finding
with absent deep tendon reflexes.
Prenatal Detection. It is possible to determine the presence of some
major open NTDs prenatally.
• Ultrasonographic scanning of the uterus and elevated maternal
concentrations of αfetoprotein
• (AFP, or MS-AFP), a fetal-specific γ-1-globulin, in amniotic fluid
may indicate anencephaly or myelomeningocele.
• Performing these diagnostic tests is between 16 and
18 weeks of gestation before AFP concentrations
normally diminish and in sufficient time to permit a
therapeutic abortion.
• Chorionic villus sampling is also a method for
prenatal diagnosis of NTDs; however, it carries
certain risks (skeletal limb depletion) and is not
recommended before 10 weeks of gestation.
Therapeutic Management
• Management of the child who has a myelomeningocele requires a
multidisciplinary team approach involving the specialties of
neurology, neurosurgery, pediatrics, urology, orthopedics,
rehabilitation, physical therapy, occupational therapy, and
social services, as well as intensive nursing care in a variety of
specialty areas. The collaborative efforts of these specialists focus
on:
• • The myelomeningocele and the problems associated with the
defect—hydrocephalus, paralysis, orthopedic deformities (e.g.,
developmental dysplasia of the hip, clubfoot), and genitourinary
abnormalities.
• • Possible acquired problems that may or may not be associated,
such as Chiari II malformation, meningitis, seizures,hypoxia, and
hemorrhage
• Other abnormalities, such as cardiac or gastrointestinal (GI)
malformations
DEVELOPMENTAL DYSPLACIA OF THE HIP
DESCRIPTION
• a spectrum of disorders related to abnormal development of the hip
that may occur at any time during fetal life, infancy, or childhood.
• gender, birth order, family history, intrauterine position, delivery
type, joint laxity, and postnatal positioning are believed to affect
the risk of DDH.
• Predisposing factors associated with it may be divided into three
broad categories:
• Physiologic factors
• Includes maternal hormone secretion and intrauterine
positioning
• Mechanical factor
• Involve breech presentation, multiple fetus,
oligohydramnios, and large infant size.
• Genetic factor
• entail a higher incidence of DDH in siblings of affected
infants and an even greater incidence of
recurrence if a sibling and one parent were affected.
DDH 2 major groups
• Idiopathic
• Infant is neurologically intact;
• Teratologic
• Involves a neuromuscular defect such as
arthrogryposis or spina bifida.
• The teratologic forms usually occur in
utero and are much less common.
Three degrees of DDH
Acetabular dysplasia
• the mildest form of DDH
• there is neither subluxation nor dislocation
• There is a delay in acetabular development evidenced by
osseous hypoplasia of the acetabular roof that is oblique and
shallow, although the cartilaginous roof is comparatively
intact
• The femoral head remains in the acetabulum.
Subluxation
• The largest percentage of DDH
• implies incomplete dislocation of the hip and is sometimes
regarded as an intermediate state in the development from
primary dysplasia to complete dislocation.
• The femoral head remains in contact with the acetabulum, but
a stretched capsule and ligamentum teres cause the head of the
femur to be partially displaced
• Pressure on the cartilaginous roof inhibits ossification and
produces a flattening of the socket.
Dislocation
• The femoral head loses contact with the acetabulum and is
displaced posteriorly and superiorly over the
fibrocartilaginous rim
• The ligamentum teres is elongated and taut.
Therapeutic Management
Newborns to Age 6 Months.
• The hip joint is maintained by dynamic splinting in a safe
position with the proximal femur centered in the acetabulum
in an attitude of flexion.
• The Pavlik harness is the most widely used.
• The harness is worn continuously until the hip is proved stable
on clinical and ultrasound examination, usually in 6 to 12
weeks.
Ages 6 to 24 Months
• A surgical closed reduction is performed, and the child is
placed in a spica cast for approximately 12 weeks.
• An abduction orthosis may be used instead of a hip spica cast
• In the event that the hip remains unstable, an open reduction is
performed
Older Children
• Operative reduction, which may involve preoperative traction,
tenotomy of contracted muscles, and any one of several
innominate osteotomy procedures designed to construct an
acetabular roof, often combined with proximal femoral
osteotomy, is usually required.
• After cast removal range-of-motion exercises help restore
movement.

CLUBFOOT
• Clubfoot is a complex deformity of the ankle and foot that
includes forefoot adduction, midfoot supination, hindfoot
varus, and ankle equinus
• Deformities of the foot and ankle are described according to
the position of the ankle and foot.
• Talipes varus— An inversion, or bending inward
• Talipes valgus —An eversion, or bending outward
• Talipes equinus —Plantar flexion, in which the toes are lower
than the heel
• Talipes calcaneus —Dorsiflexion, in which the toes are
higher than the heel
• Talipes equinovarus —Toes lower than the heel and facing
inward
• May occur as an isolated deformity or in association with
other disorders or syndromes such as chromosome
abnormalities, arthrogryposis, cerebral palsy, or spina bifida
• The precise cause of clubfoot is unknown.
• Some authorities attribute the defect to abnormal positioning
and restricted movement in utero, although the evidence is not
conclusive.
• mechanical pressures from intrauterine positioning are likely
causes of more flexible deformities
Divided into three categories:
Positional clubfoot
• also called transitional, mild, or postural clubfoot
• believed to occur primarily from intrauterine crowding and
responds to simple stretching and casting
Syndromic clubfoot(or teratologic),
• associated with other congenital anomalies such as
myelomeningocele or arthrogryposis
• a more severe form of clubfoot that is often resistant to
treatment
• usually requires surgical correction and has a high incidence
of recurrence.
Congenital clubfoot(idiopathic)
• may occur in an otherwise normal child and has a wide range
of rigidity and prognosis.
• almost always requires surgical intervention because there is
bony abnormality
Therapeutic Management
• Treatment of clubfoot involves three stages:
correction of the deformity
• maintenance of the correction until normal muscle balance is
regained
• follow-up observation to avert possible recurrence of the
deformity.
Ponseti Method
• Serial casting is begun shortly after birth.
• Weekly gentle manipulation and serial long-leg casts allow
for gradual repositioning of the foot
• The extremity or extremities are casted until maximum
correction is achieved, usually within 6 to 10 weeks
• Most of the time a percutaneous heel cord tenotomy is
performed at the end of the serial casting to correct the
equinus
• After the tenotomy, a long-leg cast is applied and left in place
for 3 weeks.
• A Denis Browne bar with Ponseti sandals or straight-laced
shoes placed in abduction is then fitted to prevent recurrence
Hypospadias, Disorders of Sex Development, and Bladder
Exstrophy
Hypospadias— Hypospadias— Hypospadias/Epispadias
 Hypospadias is a urethral defect in which the opening is on
the ventral surface of the penis rather than at the end of the
penis.
 Epispadias is a urethral defect in which the opening is on the
dorsal surface of the penis.
 In either case, the opening may be near the glans of the penis,
midway along the penis or near the base.
 If left uncorrected, the boy may not be capable of
appropriately aiming a urinary stream from a standing
position. In addition, the abnormal placement of the urethral
opening may interfere with the deposition of sperm during
intercourse, leaving the man infertile.
 Also, if left uncorrected, the boy’s self-esteem and body
image may be damaged by the abnormal appearance of his
genitalia (Pfeil & Lindsay, 2010).
 For these reasons, the defect is usually repaired sometime
after about 1 year of age.
 The goal of surgical correction for either condition is to
provide for an appropriately placed meatus that allows for
normal voiding and ejaculation.
 The meatus is moved to the glans penis and the urethra is
reconstructed as needed.
 Most repairs are accomplished in one surgery. More extensive
reconstructions may require two stages.
NURSING ASSESSMENT
 Note history of an unusual urine stream.
 Inspect the penis for placement of the urethral meatus: it may
be slightly off center of the glans or may be present
somewhere along the shaft of the penis. Inspect for chordee, a
fibrous band causing the penis to curve downward.
 Palpate for the presence or absence of testicles in thescrotal
sac, because cryptorchidism (undescended testicles) often
occurs with hypospadias, as do hydrocele and inguinal hernia.
NURSING MANAGEMENT
 The newborn with hypospadias or epispadias should not
undergo circumcision until after surgical repair of the urethral
meatus.
 In more extreme cases, the surgeon may need to use some of
the excess foreskin while reconstructing the meatus.
 Nursing management of the infant who has undergone a
hypospadias or epispadias repair focuses on providing routine
postoperative care and parent education.
Providing Postoperative Care
Postoperatively
 assess urinary drainage from the urethral stent or drainage
tube, which allows for discharge of urine without stress along
the surgical site.
 Ensure that the urinary drainage tube remains carefully taped
with the penis in an upright position to prevent stress on the
urethral incision.
 The penile dressing is usually a compression type, used to
decrease edema and bruising.
 Administer antibiotics if prescribed.
 Assess for pain,which is usually not extensive, and administer
analgesics or antispasmodics (oral oxybutynin or B & O
suppository) as needed for bladder spasms
 Double diapering is a method used to protect the urethra and
stent or catheter after surgery; it also helps keep the area clean
and free from infection. The inner diaper contains stool and
the outer diaper contains urine,allowing separation between
the bowel and bladder output.
 double-diapering technique. Change the outside (larger)
diaper when the child is wet; change both diapers when the
child has abowel movement.
Educating the Family  Note blood-tinged urine upon return from surgery, with
clearing of urine within hours to days.
 Teach the parents how to care for the catheter and drainage
system.  If orthopedic surgery is also involved in the repair due to a
malformed pubic arch, follow through with recommended
 Have parents demonstrate their ability to irrigate the catheter
positioning or bracing to prevent further separation of the
should a mucus plug occur.
pubic arch.
 Tub baths are generally prohibited until it is time to remove
the penile dressing.
 Roughhousing, ride-on toys, or any activity involving Catheterizing the Stoma
straddling is not allowed for2 to 3 weeks.  If bladder tissue is insufficient for repair, then the bladder is
Objective of surgical correction: removed and a continent urinary reservoir is created.
 Enable child to void in standing position and direct  The ureters are connected to a portion of the small intestine
stream voluntarily in usual manner that is separated from the gastrointestinal (GI) tract,thus
 Improve physical appearance of genitalia creating a urinary reservoir. The intestines are reanastomosed
 Produce a sexually adequate organ to leave the GI tract intact and separate from the GU tract. A
stoma is created on the abdominal wall; it provides access to
THERAPEUTIC MANAGEMNT the urinary reservoir.
• Objective of surgical correction:  The stoma is catheterized about four times per day to empty
• Enable child to void in standing position and direct the reservoir of urine.
stream voluntarily in usual manner  Urine from an intestine-based urinary reservoir tends to be
mucus-like and is often cloudier than urine from a urinary
• Improve physical appearance of genitalia
bladder.
• Produce a sexually adequate organ
 Teach parents the procedure for catheterizing the urinary
reservoir and instruct them to call the child’s urologist or
Disorders of Sex Development physician or nurse practitioner if signs or symptoms of UTI
• Masculinized female (female pseudohermaphrodite) - Assign occur.
gender as female; assign gender while avoiding irreversible
surgery, realizing that some children may change gender later
in life; family participation essential THERAPEUTIC MANAGEMENT
• Incompletely masculinized male (male pseudohermaphrodite) • Potential objectives of surgical correction:
- Assign gender while avoiding irreversible surgery, realizing • Preserve renal function
that some children may change gender later in life; family • Attain urinary control
participation essential • Provide adequate reconstructive repair
• True hermaphrodite (both ovaries and testes) Assign gender • Improve sexual function (especially in males)
while avoiding irreversible surgery, realizing that some
children may change gender later in life; gender assignment
depends on predominant characteristics; family participation
essential
• Mixed gonadal dysgenesis - Assign gender while avoiding
irreversible surgery, realizing that some children may change
gender later in life; gender assignment depends on
predominant characteristics; family participation essential
Exstrophy of bladder—
 Bladder exstrophy is a structural defect that requires surgical
repair and creation of urinary stoma. Eversion of posterior
bladder through anterior bladder wall and lower abdominal
wall; associated with open pubic arch (severe defect).

Providing Postoperative Care

Nursing management
 in the postoperative period focuses on preventing infection.
 Keep the infant supine and quickly change soiled diapers to
prevent contamination of the incision with stool.
 Surgical reconstruction of the bladder within the pelvic cavity
and reconstruction of a urethra are done if enough bladder
tissue is present.
 An indwelling urethral catheter or suprapubic tube will allow
urinary drainage, allowing the bladder to rest in the initial
postoperative period.
 Ensure that catheters drain freely and do not become kinked.
Sometimes tubes or catheters used in the postoperative period
require irrigation.
 Refer to the institution’s policy and the surgeon’s orders for
specifics related to urinary catheter irrigation.
 Manage bladder spasms with oxybutynin (Ditropan) or
belladonna and opioid (B & O) suppositories as ordered.