PEDIATRIC DISORDERS

Prepared by: RITCHEL P. BOLORON, RN, LPT, MAN

NDKC Clinical Instructor

OBJECTIVES:
After the class-discussion, learners should be able to:
1. describe the implications of late preterm birth;
2. explain the special problems of the preterm infant;
3. identify common nursing diagnoses for preterm infants and explain the nursing care for each;
4. explain the complications that may result from premature birth;
5. describe the characteristics and problems of the infant with postmaturity syndrome;
6. explain the effects of fetal growth restriction;
7. compare the problems of the large-for-gestational-age infant with those of the small-for-gestational-age
infant; and
8. discuss the different pediatric neurological, heart and gastrointestinal disorders.

• CARE OF HIGH RISK NEWBORNS

LATE PRETERM INFANTS
 Infants born between 34 and 36 6/7 weeks of gestation
 they have many needs that are similar to those of preterm infants.
 They are more stable than preterm infants but are physiologically and metabolically immature
and have higher mortality and morbidity rates compared with full-term infants.
 46% of late preterm infants spent some time in a special care nursery.
 Hypothermia, hypoglycemia, feeding difficulties, hyperbilirubinemia, respiratory distress, and/or
a need for a septic workup occurred in half of the infants studied (Cooper, Holditch-Davis,
Verklan et al., 2012)

Incidence
Key facts (WHO, 2018)
 Every year, an estimated 15 million babies are born preterm (before 37 completed weeks
of gestation), and this number is rising.
 Preterm birth complications are the leading cause of death among children under 5 years
of age, responsible for approximately 1 million deaths in 2015 (1).
 Three-quarters of these deaths could be prevented with current, cost-effective
interventions.
 Across 184 countries, the rate of preterm birth ranges from 5% to 18% of babies born.
 Late preterm births comprised 8.28% of births in 2011 (Hamilton, Hoyert, Martin et al.,
2013).

Etiology
• Contributing factors in late preterm birth include (Ramachandrappa & Jain, 2011):
 elective and medically indicated inductions and cesarean births
 preterm labor
 premature rupture of membranes
 preeclampsia
 multifetal pregnancies
  obesity
 assisted reproductive technology
 advanced maternal age
 inaccurate estimate of gestational age before delivery

Characteristics of Late Preterm Infants

 They are at risk for respiratory disorders
 problems with:
• temperature maintenance
• hypoglycemia
• hyperbilirubinemia
• Feeding difficulties
• acidosis
• infection (such as respiratory syncytial virus)
This is because of their immaturity (Coffman, 2009; Pappas & Walker, 2010; Ramachandrappa & Jain,
2011)
They are also at risk for long-term neurodevelopmental disorders as well as cognitive and behavioral
problems (Talge, Holzman, Wang et al., 2010)

Preterm is defined as babies born alive before 37 weeks of pregnancy are completed.
There are sub-categories of preterm birth, based on gestational age:
 extremely preterm (less than 28 weeks)
 very preterm (28 to 32 weeks)
 moderate to late preterm (32 to 37 weeks).

Nursing Considerations

Thermoregulation
 Nurses should be more vigilant in watching for thermal instability in these infants. The
temperature, as well as other vital signs, should be checked every 3 to 4 hours for the first 24
hours and then every shift, depending on need and agency policy (AWHONN, 2010;
Ramachandrappa & Jain, 2011)
 Kangaroo care (KC), a method of providing skin-to-skin contact between infants and their
parents, is often used to keep infants warm.
 A radiant warmer or an incubator may also be used if the infant cannot maintain normal
temperature.

Feedings
 Nurses should assess feeding sessions to ensure swallowing is occurring.
 have immature suck and swallow reflexes
 The football and crosscradle holds are helpful in positioning these infants at the breast.
 Urine and stool output are monitored as indications of adequate intake.
 Breastfeeding should be evaluated at least twice daily (Ramachandrappa & Jain, 2011).
 LPIs are at risk for hypoglycemia, and blood glucose measurements should be performed.
 Supplemental feedings by bottle, gavage, or use of a supplemental nursing system may be
necessary if the infant’s weight loss is greater than 10% total or more than 3% per day (Dave &
Campbell, 2009).
 have shorter awake periods, and may fall asleep during feedings before they have fed adequately
(Cleveland, 2010).
  have difficulty with latch when breastfeeding.
 low tone and weak suck may decrease the amount of milk they obtain with each suck (Walker,
2008).
 They have an increased caloric need and should be fed every 2 to 3 hours.

• PRETERM INFANTS
 also called premature infants
 are born before the beginning of the 38th week of gestation
 The word preterm is sometimes confused with the term low birth weight (LBW)

LOW BIRTHWEIGHT refers to infants weighing 5 lb, 8 oz (2500 g) or less at birth and of any gestational
age.
Very-low-birth-weight (VLBW) infants weigh 3 lb, 5 oz (1500 g) or less at birth.
Extremely-low-birth-weight (ELBW) infants weigh 2 lb, 3 oz (1000 g) or less at birth.
fetal growth restriction (FGR) are full term and have failed to grow normally while in the uterus
• Characteristics of Preterm Infants
APPEARANCE
• They appear frail and weak
• have less developed flexor muscles and muscle tone compared with full-term infants
• Their extremities are limp and offer little or no resistance when moved.
• The infant’s head is large in comparison with the rest of the body
• Preterm infants lack subcutaneous or white fat, which makes their thin skin appear red and
translucent, with blood vessels being clearly visible.
• The nipples and areola may be barely perceptible, but vernix caseosa and lanugo may be
abundant.
• Plantar creases are absent in infants of less than 32 weeks of gestation
• The pinnae of the ears are flat and soft and contain little cartilage

• Characteristics of Preterm Infants

• In the female infant, the clitoris and labia minora appear large and are not covered by the small,
separated labia majora.
• The male infant may have undescended testes, with a small, smooth scrotal sac Characteristics of

RESPIRATORY ASSESSMENT
 Infants born before surfactant production is adequate develop respiratory distress
syndrome (RDS)
 Surfactant reduces surface tension in the alveoli and prevents their
collapse with expiration. It allows the lungs to inflate with lower negative
pressure, decreasing the work of breathing.
 Preterm infants have a poorly developed cough reflex and narrow respiratory passages
 Retractions are particularly noticeable whose weak chest wall is drawn in with each
inspiration.
 Apneic spells involve absence of breathing lasting more than 20 seconds or less if
accompanied by cyanosis, pallor, bradycardia, or hypotonia (Goodwin, 2010).

Nursing Interventions
• An oxygen hood is often used for infants who can breathe independently but need extra oxygen.
• Oxygen by nasal cannula to the infant who breathes well independently.
 • Continuous positive airway pressure (CPAP) may be necessary to keep the alveoli open and
improve expansion of the lungs.
• High-frequency ventilation may be used to provide very fast respirations with less pressure and
volume.
• Arterial blood may be drawn for testing oxygen levels.
• Pulse oximetry may also be used. This method is less invasive and provides continuous
information about oxygen partial pressure (Po2) levels through sensors attached to the skin.
• The side-lying and prone positions facilitate drainage of respiratory secretions and regurgitated
feedings

Prone position increases oxygenation, enhances respiratory control, improves lung mechanics and
volume, and reduces energy expenditure (Gardner & Goldson, 2011).

Nursing Interventions
Suctioning
• The infant is suctioned only as necessary.
• Suction should be gentle to avoid traumatizing the delicate mucous membranes.
• Suctioning also provides an entry for organisms and decreases oxygenation.
• The procedure causes changes in heart rate, blood pressure, and cerebral blood flow.
• Suction should be applied for only 5 to 10 seconds at a time and increased oxygen should be
provided before and after each suction attempt.
• The mouth is suctioned before the nose because stimulation of the nares causes reflex inspiration
that could cause aspiration of fluids in the infant’s mouth (Gardner, EnzmanHines, & Dickey,
2011b).

Thermoregulation

Problems with Fluid and Electrolyte Balance

 Preterm infants lose fluid very easily, and the loss increases with the degree of prematurity.
 The rapid respiratory rate and the use of oxygen increase fluid losses from the lungs.
 Their thin skin has little protective subcutaneous white fat and is more permeable than the skin of
term infants.
 Heat from phototherapy lights causes even more fluid to be lost through the skin.
 The ability of the kidneys to concentrate or dilute urine is poor, causing a fragile balance between
dehydration and overhydration.
 Normal urinary output is 1 to 3 mL/kg/hr for preterm infants for the first few days.
 After 24 hours of life, output less than 0.5 mL/kg/hr is considered oliguria (Blackburn, 2013).

Preterm infants need higher intakes of sodium because the kidneys do not reabsorb it well.
If they receive too much sodium, however, they may be unable to increase sodium excretion
adequately and are susceptible to sodium and water overload.

NURSING INTERVENTIONS
 Monitoring intake and output of fluids helps determine fluid balance.
 Several methods of measuring urine output:
o Plastic bags that adhere to the perineum are not suitable for the preterm infant
because they may damage the fragile skin.
o Weighing diapers is less harmful to the infant. The weight of dry diapers is
subtracted from the weight of wet diapers to determine the amount of urine
excreted.
o One gram (1 g) is equivalent to one milliliter (1 mL) of urine
o The specific gravity should range between 1.002 and 1.01 (Jones, Hayes,
Starbuck et al., 2011).
Weight.
 Changes in the infant’s weight can give an indication of fluid gain or loss .
 The undressed infant should be weighed at the same time each day with the same scale.
 Very small infants are often placed in a bed with a scale so that they are not disturbed for daily
weighing.
 They may be weighed two to three times a day to monitor their fluid status more closely.
Signs of Dehydration or Overhydration.
 Observe for signs that indicate the infant has received too little or too much fluid.
The nurse must carefully regulate intravenous (IV) fluids using infusion control devices that administer
fluid with a precision of 0.1 mL/hr to help prevent fluid volume overload (Jones et al., 2011).

Problems with Infection

Factors that contribute to the high rate of infection include:
 exposure to maternal infection
 lack of adequate passive immunity from the transfer of
immunoglobulin G (IgG) from the mother during the third trimester
immature response to infection.
 prolonged stay in the hospital increases the likelihood of acquiring an infection from
multiple exposures to organisms

Nursing Interventions:
 Handwashing
 Scrupulous cleanliness and maintenance of the infant’s skin integrity. Even normal flora
on the hands of caregivers may cause sepsis.
 Parents and staff members should thoroughly wash their hands and arms before handling
infants.
  No jewelry is worn in many units because of the possibility of it carrying organisms.
 Exposure to family and staff members who have contagious diseases should be
prevented.

Problems with Pain

• It was once thought that newborns, particularly preterm infants, were neurologically too immature
to feel pain.
• It is now recognized that preterm infants do feel pain and that pain stimuli cause physiologic and
behavioral changes in infants.
• Pain is the fifth vital sign, pain assessment is performed whenever vital signs are taken.

NURSING INTERVENTIONS
 Nurses should prepare infants for potentially painful procedures by waking them slowly
and gently and using containment.
 KC and breastfeeding also are used to reduce pain. They are a way for mothers to be
involved in helping relieve infant pain.
 The mother’s voice and smell, which are familiar to the infant, are added to skin-toskin
contact to increase pain relief (Campbell-Yeo, Fernandes, & Johnston, 2011).
 Handling before a painful procedure should be minimized, if possible.

Containment simulates the enclosed space of the uterus, prevents excessive and disorganized motor
activity, and is comforting to infants.
It involves keeping the extremities in a flexed position with swaddling, positioning devices, or with the
nurse’s hands.
The infant is in the supine or side-lying position with at least one of the infant’s hands near the mouth
for sucking.
Containment is also called facilitated tucking (Fernandes, Campbell-Yeo, & Johnston, 2011).

• POSTTERM INFANTS
• Postterm infants are those who are born after the 42nd week of gestation.

 Approximately 3% to 14% of all pregnancies are considered postterm (V. C. Smith,

2012).
 In some cases, the fetus continues to be well supported by the placenta and is of normal
size or is large for gestational age.
 Some grow to more than 8 lb, 13 oz (4000 g), placing them at risk for birth injuries or
cesarean birth.
 Placental insufficiency is present
 Decreased amniotic fluid volume (oligohydramnios)
 Compression of the umbilical cord may occur.

 The fetus may not receive the appropriate amount of oxygen and nutrients and may be
small for gestational age.
 This condition results in hypoxia and malnourishment in the fetus and is called
postmaturity syndrome or dysmaturity syndrome.
 The fetus may pass meconium as a result of hypoxia before or during labor, increasing
the risk of meconium aspiration at delivery
 • Assessment (Postterm Infants):

• Most infants will be normal at birth.

• If the infant is large, the nurse should observe for injury and hypoglycemia.
• The infant with postmaturity syndrome may have an apprehensive look associated with hypoxia
(McGrath & Hardy, 2011).
• The infant may be thin with loose skin and little subcutaneous fat.
• There is little or no vernix caseosa.
• the infant generally has abundant hair on the head and long nails.
• The skin is wrinkled, cracked, and peeling.
• If meconium was present in the amniotic fluid, the cord, skin, and nails may be stained yellow
green, indicating that meconium was present for some time.
• The umbilical cord is thin with little Wharton’s jelly.

What are the complications of being small for gestational age?

• Lower oxygen levels than normal.

• Low Apgar scores.
• Breathing in the first stools (meconium) passed in the womb. This can cause
breathing problems.
• Low blood sugar.
• Difficulty keeping a normal body temperature.
• Too many red blood cells.

Delivery complications of LGA

Vaginal delivery may be difficult and occasionally results in birth injury, particularly including:

 Shoulder dystocia
 Fracture of the clavicle or limbs
 Perinatal asphyxia

Other complications occur when weight is > 4000 g.

There is a proportional increase in morbidity and mortality due to the following:
 Respiratory distress
 Meconium aspiration
 Hypoglycemia
 Polycythemia

SPINA BIFIDA
 Congenital defect of the spinal/neural tube in which there is an incomplete closure of the spinal
column due to one or two missing vertebral arches
th
 Usually occurs during 4 week of embryonic life but the exact cause is unknown.
  Deficiency in Folic Acid of the mother during the pregnancy and environmental factors are
thought to be the cause of spinal bifida.

CLASSIFICATIONS
1. SPINA BIFIDA OCCULTA
 Seen as a small dimple at the lower back, usually asymptomatic and creates health
problems, often no treatment is needed.
2. MENINGOCELE
 Sac-like cyst that contains meninges and spinal fluid that protrudes through the defect
3. Meningomyelocele
 With herniated sac of meninges, spinal fluid and a portion of the spinal cord and its
nerves, which protrude through the defect in the spine
 It is the most severe form.

ASSESSMENT FINDINGS
 Visible sac-like structure or dimpling of the skin at any point of the spinal column.
 Associated defects/problems found in meningomyelocele
 Hydrocephalus
 Bowel/bladder dysfunction
 Paralysis of lower extremities
 Associated meningitis

SURGICAL MANAGEMENT
• Currently, it is done as soon after birth as possible (usually within 24 to 48 hours) so infection
through the exposed meninges does not occur.

NURSING MANAGEMENT
 Women who have had one child with a spinal cord disorder are advised to have a
maternal serum assay or amniocentesis for AFP levels to determine if such disorder is
present in a second pregnancy (levels will be abnormally increased if there is an open
spinal lesion).
 Evaluate sac and lesions
 Perform neurological assessment
 Monitor ICP
 Measure head circumference, assess anterior fontanels for fullness protect the sac, cover
with a sterile, moist non-adherent dressing
 Place in a prone position to minimize tension on the sac and the risk of trauma
 Use aseptic technique to prevent infection
 Assess the sac for redness, clear or purulent drainage abrasion, irritation, and signs of
infection
 Administer antibiotics as prescribed
 Administer anti-cholinergics to improve urinary continence and laxatives to achieve
bowel continence.

PREVENTION
 Pregnant women are advised to ingest 600 micrograms of folic acid daily to help prevent
these disorders during the first trimester.
 HYDROCEPHALUS

 Excess of CSF in the ventricles of the subarachnoid space

 Risk factors include:
 Infant meningitis/encephalitis
 Hemorrhage of Tumor – clock passage of fluid
 Arnold-chiari disorder – elongation of the lower brainstem and
th
displacement of the 4 ventricle into upper cervical canal.
 Surgery for meningocele – portion of subarachnoid space is removed
causing less surface area for absorption of CSF

TYPES OF HYDROCEPHALUS
1. COMMUNICATING
 Occurs as a result of impaired absorption within the subarachnoid space

2. NON-COMMUNICATING
 Obstruction of cerebrospinal flow within the ventricular system
CAUSES OF EXCESS CSF
st nd
 OVERPRODUCTION of fluid by choroids plexus in 1 or 2 ventricle.
 OBSTRUCTION of the passage of fluid in narrow aqueduct of sylvius (most common)
 Interference with the absorption of CSF from subarachnoid pace

ASSESSMENT FINDINGS
 ANTERIOR FONTANEL BULGING
Macewen’s sign – bones of the head are widely separated that
produces a cracked pot sound upon percussion
Bossing sign – brow bulges

• TREATMENT: SURGICAL SHUNTING

 VENTRICULOPERITONEAL (V-P) SHUNT
 Connects the lateral ventricle of the brain to the peritoneal cavity
 Most commonly used shunt in children.
 ATRIOVENTRICULAR (A-V) SHUNT
 Connect the lateral ventricle to the right atrium of the heart.
 VENTRICULOURETERAL (V-P) SHUNT
 Connects the lateral ventricle of the brain to the ureter, requires unilateral
nephrectomy which may cause recurrent fluid and electrolyte imbalance.
NURSING CARE
 Assess daily head circumference
 Protect the child’s head
 Maintain nutrition and hydration

PREOPERATIVE INTERVENTIONS
 Monitor intake and output, give small frequent feedings as tolerated until a preoperative NPO
status is prescribed.
  Reposition head frequently and use an egg crate mattress under the head to prevent pressure
sores.
 Prepare the child and family for diagnostic procedure and surgery.

POSTOPERATIVE INTERVENTIONS
 Monitor vital signs and neurological signs
 Position the child on the unoperated side to prevent pressure on the shunt valve.
 Keep the child flat as prescribed to avoid rapid reduction of intracranial fluid.
 Observe for increased ICP, if increased ICP occurs, elevate the head of the bed to 15 to
30 degrees to enhance gravity flow through the shunt.
 Monitor for signs of infection and assess dressings for drainage.
 Measure head circumference.
 Monitor intake and output.
 Provide comfort measures.
 Administer medications as prescribed, which may include diuretics, antibiotics or
anticonvulsants.

• BACTERIAL MENINGITIS AND ENCEPHALITIS

 An acute bacterial infection of the CNS that may be acquired as the primary disease or as a result
of a local infection, systemic infections, trauma and neurosurgery.

CAUSES:
 Bacterial meningitis (Haemophilus influenza type B, Streptococcus pneumoniae, or Neisseria
meningitides) occurs in epidemic form and can be transmitted by droplets from nasopharyngeal
secetions.
 Viral meningitis is associated with viruses such as mumps, paramyxovirus, herpesvirus, and
enterovirus.

MENINGITIS is an inflammation of membranes surrounding the brain and spinal cord.

ENCEPHALITIS is an inflammation of the brain itself.

ASSESSMENT FINDINGS:
 Opisthotonus position
 Stiff neck and nuchal rigidity
 (+) Kernig’s sign
 Inability to extend the leg when the thigh is flexed anteriorly at the hip.
 (+) Brudzinski sign
 Neck flexion causes adduction and flexion movements of the lower extremities
DIAGNOSTIC PROCEDURE

 Lumbar puncture
 Clouding of CSF, increased protein and decreased Glucose
 Smear and culture of CSF and blood demonstrate the presence of organism

PHARMACOLOGIC THERAPY
 Antibiotic Therapy
 IV Penicillin G (drug of choice)

NURSING INTERVENTIONS
 Isolate infant.
 Ensure patent airway and promote safety during seizures.
 Monitor and control temperature.
 Perform neurological assessment and monitor her seizures
 Assess for the complication of inappropriate Antidiuretic Hormone (ADH) secretion causing fluid
retention (cerebral edema) and dilutional hyponatremia
 Assess for changes in level of consciousness and irritability
 Monitor intake and output
 Assess nutritional status.
 Determine close contacts of the child with meningitis because the contacts will need prophylactic
treatment.

• CEREBRAL PALSY
 A neuromuscular disorder characterized by lack of control of the voluntary muscles, abnormal
muscle tone and incoordination

• THREE TYPES OF CEREBRAL PALSY

a.) SPASTIC (most common)
The cortex is affected resulting in the child having a scissor-like gait where one foot crosses in
front of the other foot.

b) ATHETOID/ DYSKINETIC
The total basal ganglia are affected resulting in uncoordinated involuntary motion.

c) ATAXIC
The cerebellum is affected resulting in poor balance and difficulty with muscle coordination

ASSESSMENT FINDINGS:
• Infant may manifest early signs of cerebral palsy
  Abnormal posturing
 Difficulty feeding
 Tremors/Seizures
 Persistence of primitive reflexes

• DOWN SYNDROME
 A chromosomal aberration characterized by Trisomy 21 or the tripling of chromosome 21
 Presence of an extra copy of chromosome 21 resulting in a total number of 47 rather than 46
 It is found to be more common among children of mothers with increased of advances age

• ASSESSMENT FINDINGS
 Facial characteristics:
 wide gap between the eyes
 flat nose
 large tongue

• ASSOCIATED PROBLEMS AND FINDINGS

 Intelligence:
 varies from severely retarded to below normal (IQ less than 20 to IQ between 50-70)

 Congenital Anomalies:
 VSD, Hirchsprung’s disease and Leukemia

 Growth is reduced with rapid weight gain

 Sexual development may be delayed, incomplete or both

Head characteristics:
 Flattened posterior and anterior surfaces of the skull
 obviously flat occiput

Extremities:
 Simian crease
 Abnormal horizontal line on the palm of the hands
 plantar furrow
 vertical line on the sole
 first and second toes widely spaced

Brushfield’s spots
 White specks in the iris of the eye
 Low set ears

NURSING INTERVENTIONS
 Goal for the child is to reach his optimum development potential and be able to cope as effectively
as possible to this mental handicap
 Treatment is based on the child’s developmental age rather than chronological age
 Emphasize the importance of providing the child consistent care to favor the development of trust
(foundation of personality) and feeling of security

REYE’S SYNDROME
 Is an acute encephalopathy that follows viral illness and is characterized pathologically by
cerebral edema and fatty changes in the liver.
Definitive Diagnosis: Liver biopsy
Etiology: Unknown
INTERVENTIONS:
 Provide rest and decrease stimulation in the environment
 Assess neurological status.
 Monitor for altered LOC and signs of increased Intracranial Pressure (ICP)
 Monitor liver function studies
 Monitor intake and output.
 Monitor for signs of bleeding and signs of impaired coagulation.

SEIZURE DISORDERS

 Excessive and unorganized neuronal discharges in the brain that activate associated motor and
sensory organs.
 Classified as generalized, partial or unclassified, depending on the area of the brain involved.

Diagnostic Tests:
• Electroencephalography (EEG), a test that records electrical activity in your child's brain using
tiny wires attached to the head (EEG is highly sensitive and can pick up even small seizures that
don't lead to physical symptoms.)
• Magnetic resonance imaging (MRI), uses powerful magnets and radio waves to create a
detailed view of your brain.
• Computerized tomography (CT), uses X-rays to obtain cross-sectional images of your brain
• Positron emission tomography (PET), uses a small amount of low-dose radioactive material
that's injected into a vein to help visualize active areas of the brain and detect abnormalities.
SEIZURE PRECAUTIONS:
 Raise side rails when child is sleeping or resting
 Pad side rails and other hard objects
 Place waterproof mattress or pad on bed or crib.
 Instruct child to wear or carry medical identification
 Instruct child precautions to take during potentially hazardous activities.

INTERVENTIONS FOR SEIZURE:

 Ensure airway patency.
 Have suction equipment and oxygen available.
 Time the seizure episode.
 If the child is standing or sitting, ease the child’s head; if no bedding is available, place your own
hands under the child’s head or place the child’s head in your own lap.
 Loosen restrictive clothing
 Clear the area of any hazards or hard objects.
 Allow the seizure to proceed and end without interference.
 If vomiting occurs, turn the child to one side as a unit
 Do not restrain the child, place anything in the child’s mouth, or give any food or liquids to the
child.
 Remain with the child until the child recovers.
 Observe for the incontinence which may occur during the seizure
CONGENITAL HEART DISEASE

 An abnormality of structure or function of the heart, circulatory system or both

 Usually result from an alteration or failure of development of a structure of the heart

ASSESSMENT FINDINGS
 Tachycardia, tachypnea
 Profuse scalp sweating
 Fatigue
  Irritability
 Respiratory distress
 Feeding problem
 Poor weight gain/ failure to thrive
 Frequent respiratory infections; cough

• ACYANOTIC HEART DEFECTS

ACYANOTIC HEART DISORDERS
 heart or circulatory anomalies that involve either a stricture to the flow of blood or a shunt that
moves blood from the arterial to the venous system (oxygenated to unoxygenated blood, or left-
to-right shunts)
 VSD ventricular septal defect
 ASD atrial septal defect
 PDA patent ductus arteriosus
 PS pulmonary stenosis
 COA coarctation of aorta

• ACYANOTIC HEART DEFECTS

ASSESSMENT FINDINGS
 audible murmurs – most common sign
 Loud systolic murmur – VSD
 Machinery murmur – PDA

 Easy fatigability shown as brow sweating (during feeding or crying episodes)

 Hepatomegaly due to backup of blood
COMPLICATIONS
 Congestive Heart Failure (most common)
 Respiratory distress manifested by :
 Cough
 Diaphoresis
 Severe dyspnea

• VENTRICULAR SEPTAL DEFECT

 Almost always left to right shunt
 Abnormal opening between the left and right ventricles
 Most common CHD 30%
 Overloading of Right Ventricle and pulmonary circulation
 Pulmonary hypertension and Right ventricular failure may occur
 Reverse shunt (right to left) may develop Eisenmenger’s syndrome (pulmonary hypertension)

• ATRIAL SEPTAL DEFECT

 Abnormal opening between the Atria that causes an increased flow of oxygenated blood into the
right side of the heart.
 Failure of the atrial septum to close

THREE (3) MAJOR TYPES

ASD 1 – Ostiumprimum ( opening at the lower end of the septum)
ASD 2 – Ostium Secondum (opening near the center of the septum)
ASD 3 – Sinus Venosus Defects (Opening near the junction of the SVC and the right atrium)

ATRIAL SEPTAL DEFECT

 Asymptomatic if small defect
  Systolic ejection murmurs
 Growth retardation (slow weight gain)
 Heart failure symptoms usually occur in ostium primum defects
 Right ventricular hypertrophy
 Frequent respiratory infections, dyspnea
 Easy fatigability

Nursing Interventions
 Provide family teaching about treatment options
 Small defect spontaneously close
 Non-surgical treatment: the defect may be closed by using devices during a cardiac
catheterization
 Defect are usually repaired in girls due to possibility of pulmonary hypertension
during pregnancy.
Surgical treatment:
 open repair with cardio pulmonary bypass before school age
• PATENT DUCTUS ARTERIOSUS
• Results when the fetal ductus arteriosus fails to close completely after birth

CLINICAL FINDINGS:
 Asymptomatic if defect is small
 Loud MACHINE-LIKE murmur
 Frequent respiratory infection
 Splenomegaly, poor weight gain, Tachypnea and irritability
 Widening pulse pressure

MEDICAL MANAGEMENT:
 INDOMETHACIN (Indocin)
 A prostaglandin inhibitor, maybe administered to close a patent ductus in premature
infants and some newborns.

NURSING INTERVENTIONS: (PDA)

• Some PDAs close spontaneously
• Premature infants Prostaglandin Synthetase Inhibitors
 stimulates closure of ductus
• Management of the defect may be closed during cardiac catheterization or the defect may require
surgical management.

• COARCTATION OF THE AORTA

 Restricted lumen of the aorta proximal to, at or distal to the ductus arteriosus.
 Localized narrowing of the aorta

ASSESSMENT FINDINGS:
 Elevated upper body blood pressure produces headache and vertigo
 Pathognomonic sign: Bounding radial pulse and absent femoral pulse
 Epistaxis, headache, fainting, lower leg cramps
 Systolic murmur
 • Treatment: (COARCTATION OF THE AORTA)
 Children with this disorder require therapy with digoxin and diuretics
 It aims to reduce the severity of the congestive heart failure from hypertension.
 Interventional Angiography (a balloon catheter or surgery)
 With surgery, the narrowed portion of the aorta is removed and the new ends of the aorta are
anastomosed.

• PULMONARY STENOSIS
 PULMONARY STENOSIS is narrowing at the entrance to the pulmonary artery
 Resistance to blood flow causes right ventricular hypertrophy and decreased pulmonary blood
flow, the right ventricle may be hypoplastic
 Pulmonary Atresia – is the extreme form of pulmonary stenosis in that there is total fusion of
the commissures and no blood flows to the lungs
ASSESSMENT FINDINGS:
 A characteristic murmur is present.
 The infant or child may be asymptomatic
 Newborns with severe narrowing will be cyanotic
 If pulmonary stenosis is severe, Congestive Heart Failure occurs
 Signs and symptoms of decreased cardiac output may occur.

MEDICAL MANAGEMENT:
 Nonsurgical treatment is done during cardiac catheterization to dilate the narrowed valve.

SURGICAL MANAGEMENT:
 In INFANTS: Transventricular (closed) Valvotomy procedure
 In CHILDREN: pulmonary valvotomy with cardiopulmonary bypass

CYANOTIC CONGENITAL HEART DISEASES

- Tetralogy og Fallot
- Truncus Arteriosus
- Tricuspid Atresia
- Transposition of Great Vessels

ASSESSMENT FINDINGS:
• Central cyanosis
• Recurrent respiratory infections
• Easy fatigability
• Retarded physical growth
• Clubbing of fingers
• Squatting in older children
• Increase hematocrit

COMPLICATIONS:
 Brain infarctions and blood clots
 Left sided heart failure
• TETRALOGY OF FALLOT
• A combination of 4 defining features
  Pulmonary Stenosis
 Right Ventricular Hypertrophy
 Overriding Aorta
 VSD
ASSESSMENT FINDINGS
o Infant – cyanotic at birth or may have mild cyanosis over the first year of life
o TET SPELLS
o irritability
o pallor
o Blackouts or convulsions
o cyanosis at rest
o Squatting
o Slow weight gain
o Exertional dyspnea, fatigue, slowness due to hypoxia

NURSING INTERVENTIONS
 Maintain respiration/oxygenation
 Proper positioning
 Cyanotic child: lateral position , knee-chest, squatting for preschool or older children
 Promote Rest
 Keep warm
 Prevent infection
 Administer drugs as ordered: digitalis and diuretics

What can be done about tetralogy of Fallot?

 Tetralogy of Fallot is treated surgically.
 A temporary operation may be done at first if the baby is small or if there are other problems.
 Complete repair comes later.
 Sometimes the first operation is a complete repair.
Temporary Operation
• In some infants, a shunt operation may be done first to provide adequate blood flow to the lungs.
• This is not open-heart surgery and doesn't fix the inside of the heart.
• The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta)
and the pulmonary artery.
• The shunt is closed when a complete repair is done later.

Complete Repair
• Complete repair tends to be done early in life.
• The surgeon closes the ventricular septal defect with a patch and opens the right ventricular
outflow tract by removing some thickened muscle below the pulmonary valve, repairing or
removing the obstructed pulmonary valve and, if needed, enlarging the branch pulmonary arteries
that go to each lung.
 Rastelli Repair
• It is similar to the type of repair used for some other heart defects.
• It is when a tube is placed between the right ventricle and the pulmonary artery.
• TRICUSPID ATRESIA
 Is an extremely serious disorder because the tricuspid valve is completely closed, allowing no
blood to flow from the right atrium to the right ventricle.
 As long as the foramen ovale and ductus arteriosus remain open, the child can obtain adequate
oxygenation.

ASSESSMENT:
 Cyanosis
 Tachycardia
 Dyspnea
 Older children: chronic hypoxemia and clubbing
TREATMENT:
• Surgery consists of the construction of a vena cava-to-pulmonary artery shunt, which deflects
more blood to the lungs, or a Fontan Procedure (sometimes termed as a Glenn Shunt Baffle),
which restructure the right side of the heart.

NURSING INTERVENTIONS:
 An infusion of Prostaglandin E1 (PGE1) is begun to ensure that the ductus remains open

Gastrointestinal Tract Disorder

• Cleft Lip and Palate
• These are among the most common congenital anomalies.
• They occur together or separately on one or both sides.
Lip: minor notching of the lip or complete separation through the lip and into floor of nose.
Palate: only the soft palate or division of the entire hard and soft palate. Both genetic and environmental
factors are included in the causes.

Causes:
Genetic, hereditary
Environment – exposure to radiation
Rubella virus
chromosome abnormalities
teratogenic factors

Assess: Ability to suck or swallow

Therapeutic Management
 Lip surgery is usually performed by 3 months to enhance appearance and parental bonding.
 Further surgery may be needed at 4 to 5 years.
 Palate surgery is usually done by 1 year to minimize speech problems.
 Long-term follow-up is necessary for orthodontia, speech therapy, and treatment of possible
hearing problems.
 Orthodontia- the treatment of irregularities in the teeth (especially of alignment and occlusion)
and jaws, including the use of braces.

Nursing Considerations:
The degree of the cleft determines the approach to feeding. Experiment to find methods that work best
for individual infants. Try:
1. Breastfeeding (soft breast tissue fills in a small cleft of the lip or palate)
2. Nipples with enlarged hole
3. Compressible bottles
4. Special longer nipples
5. Special assistive devices
 Feed the infant in the upright position because milk enters nasal passages through the
palate, causing an increased tendency to aspirate.
 Feed slowly with frequent stops to burp because infants tend to swallow excessive air.
 Help the parents deal with their disappointment over the infant with an obvious anomaly.
 Show them before and after pictures of plastic surgery.
 Reinforce the physician’s explanation of plans for surgery.
 Teach parents feeding techniques.
6. Prevent infections. Infants are especially susceptible to respiratory tract and ear infections, which can
delay surgery. Ear infections may lead to hearing loss

• POSTOPERATIVE INTERVENTION
CLEFT LIP REPAIR ( CHEILOPLASTY)
 Closure of cleft lip defect precedes that of the cleft palate and is usually performed during the
first weeks of life.
 A lip protector device maybe taped securely to the cheeks to prevent trauma to the suture
line.
 Position the child on the side opposite to the repair or on the back
 Avoid prone position to prevent rubbing of the surgical site on the mattress.
 After feeding, cleanse the suture line with plain normal saline solution.
 Prevent the child from crying if possible
 Do not let the LOGAN’s Bar get wet.

• POSTOPERATIVE INTERVENTION
CLEFT PALATE REPAIR (PALATOPLASTY)
 Clift palate repair is performed sometimes between 12 to 18 months of age to allow for the palatal
changes that take place with normal growth.
 A cleft palate is closed before the child develops faulty speech habits.
 Child is allowed to lie on the abdomen (prone).
 Feeding are resumed by bottle, breast or cup
 Oral packing maybe secured to the palate
 Do not allow child to brush teeth
 Avoid hard foods
 Soft Elbow or Jacket restraints maybe used to keep the child from touching the repair site.
 Avoid contact with sharp objects near the repair site
 Avoid oral suction or placing objects in the mouth.
• In esophageal atresia (EA) the esophagus is most commonly divided into two unconnected
segments (atresia) with a blind pouch at the proximal end.
• If the distal end is connected to the trachea, it causes tracheoesophageal fistula (TEF).
• The cause is a failure of normal development during the fourth week of pregnancy.
 Esophageal Atresia and Tracheoesophageal Fistula

 In esophageal atresia (EA) the esophagus is most commonly divided into two
unconnected segments (atresia) with a blind pouch at the proximal end.
 If the distal end is connected to the trachea, it causes tracheoesophageal fistula (TEF).
 The cause is a failure of normal development during the fourth week of pregnancy.

Clinical Assessment:
• polyhydramnios occurs because the excessive fluid may be caused by fetal inability to swallow
amniotic fluid
• excessive frothy drooling and needing suction more often than usual
• regurgitation occurs from secretions that pool in a blind pouch
• a catheter will not pass into the stomach.
• If a fistula connects the distal esophagus and the trachea, the stomach becomes distended with air
from the trachea.
• Gastric secretions may be aspirated into the lungs, causing a severe inflammatory reaction.

Therapeutic Management
 Diagnosis is confirmed by symptoms and radiography.
 Esophageal suctioning should be used for the upper pouch, and a gastrostomy tube is placed.
 Surgery involves ligation of the fistula and anastomosis of the esophageal segments.
 If the separation is large, surgery may be done in stages to allow growth.
 Long-term follow-up is needed for esophageal reflux and dilation of strictures that may form at
the surgical site.

 Prevent aspiration by maintaining the infant in a semi-upright position to prevent reflux

of gastric fluids.
 Maintain suction equipment.
 Care after surgery involves low-pressure ventilation, chest tubes, parenteral nutrition, and
gastrostomy feedings.

Omphalocele and Gastroschisis

• Both of these anomalies are caused by congenital defects in the abdominal wall.
In omphalocele, the intestines protrude into the base of the umbilical cord.

Therapeutic Management
 A gastric tube is placed to decrease air in the stomach.
 Gastric suction, parenteral nutrition, and antibiotics are necessary.
 Surgery is performed as soon as the infant is stable.
 A Silastic silo (pouch) may be used to replace the intestines gradually over a period of days to
prevent pressure on the other organs.
 The abdomen is closed when the contents have been replaced in the abdominal cavity.

Nursing Considerations
 Place the infant’s torso into a sterile plastic bag or cover the intestines with Silastic or warm sterile
saline dressings wrapped with plastic immediately after birth to reduce heat and water loss.
  Observe for respiratory distress from increased intraabdominal pressure.
 Prevent infection and trauma.
 Position to avoid pressure on the intestines.

• Diaphragmatic Hernia
• The diaphragm fails to fuse during gestation.
• A large or small part of the abdominal contents moves into the chest cavity, usually on the left
side.
• If the herniation is large enough, the lungs may fail to develop (hypoplastic lungs).
• When gas fills the bowel, further pressure on the heart and lungs results.

Clinical Assessment
• Mild to severe respiratory distress may occur at birth, with breath sounds diminished or absent
over the affected area, and barrel chest.
• The heartbeat may be displaced to the right. Bowel sounds are heard in the chest.
• The abdomen may be scaphoid (concave).
• The condition may be diagnosed prenatally by ultrasound or by radiography after birth.

Therapeutic Management
 Fetal surgery may be performed.
 An endotracheal tube is placed for mechanical ventilation and a gastric tube for
decompression of the stomach.
 Surgery to replace the intestines and repair the defect is performed when the infant is
stable.
 Extracorporeal membrane oxygenation (ECMO) or inhaled nitric oxide may be used.

Nursing Considerations
 Position the infant on the affected side to allow the unaffected lung to expand.
 Elevate the head to decrease pressure on the heart and lungs. Assist with ventilation, and
monitor respiratory status.
 Continue to monitor respiratory status after surgery to determine whether lung function
will be adequate.

• PYLORIC STENOSIS
 Hypertrophy of the circular muscles of the pylorus causes narrowing of the pyloric canal between
the stomach and the duodenum.
 Stenosis develops in the first few weeks of life.

Assessment Findings:
 With the condition at 4 to 6 weeks of age, infants begin to vomit almost immediately
after each feeding.
 Peristaltic waves are visible from left to right across the epigastrium during or
immediately following a feeding.
 Vomiting grows increasingly forceful until it is projectile.
 Fluid is unable to pass easily through the stenosed and hypertrophied pyloric valve.
Clinical Manifestations:

 Vomiting- projectile, non-bilious

 Hunger and irritability
 Olive shaped mass in the epigastrium just right of the umbilicus
 Dehydration and malnutrition
 Electrolyte imbalance

• SURGICAL MANAGEMENT
 Prepare the child for Pyloromyotomy
 An incision through the muscle fibers of the pylorus that may be
performed by laparoscopy.

NURSING INTERVENTIONS:
 Monitor vital signs; intake and output and weight
 Monitor for signs of dehydration and electrolyte imbalance
 Oral feedings are withheld to prevent further electrolyte depletion.
 An infant who is receiving only IV fluid generally needs a pacifier to meet nonnutritive
sucking needs.

• LACTOSE INTOLERANCE
 Inability to tolerate lactose as a result of an absence or deficiency of lactase; an enzyme found in
the secretions of the small intestine that is required for the digestion of lactose.

NURSING INTERVENTIONS:
 Eliminate the offending dairy products
 Provide information to the parents about enzyme tablets that predigest the lactose in milk or
supplement the body’s own lactate.
 Substitute soy-based formulas for cow’s milk formula or human milk.
 Provide calcium and Vitamin D supplements to prevent deficiency
 Limit milk consumption to one glass at a time
 Instruct the child and family that the child should drink milk with other foods rather than by itself.
 Encourage consumption of hard cheese, cottage cheese, or yogurt which contains the inactive
lactase enzyme.
 Encourage consumption of small amounts of dairy foods daily to help colonic bacteria adapt to
ingested lactose.
 Instruct the parents about the foods that contain lactose including hidden sources.

• CELIAC DISEASE
(gluten-induced enteropathy or celiac sprue)
 Intolerance to gluten, protein component of:
B – arley
R – ye
O – ats
W –heat
 Results in the accumulation of the amino acid glutamine, which is toxic to intestinal mucosal
cells.
 Intestinal villi atrophy occurs, which affects absorption of ingested nutrients.
 There is usually an interval of 3 to 6 months between the introduction of gluten in the diet and the
onset of symptoms.
Assessment Findings:
Acute or infectious diarrhea
Steatorrhea
Anorexia
Abdominal pain and distention
Muscle wasting, particularly in the buttocks and extremities
Vomiting
CELIAC CRISIS
 Precipitated by infection, fasting, ingestion of gluten
 Can lead to electrolyte imbalance, rapid dehydration, severe acidosis
 Causes profuse watery diarrhea and vomiting

NURSING INTERVENTIONS:
 Maintain a gluten-free diet, substituting corn, rice and milled as grain sources
 Instruct parents and child about lifelong elimination of gluten sources such as wheat, rye,
oats and barley.
 Administer mineral and vitamin supplements including iron, folic acid , and fat-soluble
supplements A, D, E, K

FOOD ALLOWED:
 Beef
 Pork
 Fish, eggs, milk and dairy products
 Vegetables, fruits, rice, corn, gluten-free wheat flour, cornflakes

FOOD PROHIBITED:

o Commercially prepared ice cream

o Malted milk
o Grains: wheat, rye, barley such as:
o Breads
o Cookies, cakes, crackers, cereals , spaghetti, macaroni, noodles

• DUODENAL ATRESIA
 Congenital absence or complete closure of a portion of the lumen of the duodenum

Assessment Findings:
 Early bilious vomiting
 No abdominal distention
 Continued vomiting even when infant has not been feed for several hours
 Absent bowel movements after few meconium stool usually confirmed by radiography
 X-Ray of the Abdomen: two large air filled spaces, the so called “double bubble sign”
 • HIRSCHPRUNG’S DISEASE
 the disease occurs as the result of an absence of ganglion cells in the rectum and other areas of
the affected intestine.
 Congenital anomaly also known as congenital aganglionosis or aganglionic megacolon.

Assessment Findings:

 “RIBBON-LIKE STOOLS”
 Shows symptoms in the first 6 weeks of life
 No bowel movement in the first 48 hours of life
 Gradual bloating of the abdomen
 Gradual onset of bile stained vomitus
 Fecal odor of breath
 Loss of appetite
 Delayed growth
 Anemia
 Passing small, watery stool

• Diagnosis
• Abdominal X-Ray
• Barium enema
• Biopsy of the rectum or large intestine

NURSING INTERVENTION (POST-OP):

 Measure abdominal girth daily
 Assess the surgical site for redness, swelling and drainage
 Assess the stoma if present for bleeding or skin breakdown (stoma should be red and moist)
 Maintain NPO status until bowel sounds return or flatus passed, usually within 48 to 72 hours
 Maintain the NGT to allow intermittent suction until peristalsis returns
 Maintain IV fluids until the child tolerates appropriate oral intake
 Provide parents with instructions regarding ostomy care.

MEDICAL TREATMENT SURGERY:

 Bowel resection
 Abdomino-perineal pull through by about one year
o Maintain low fiber, high-calorie, high-protein diet, parenteral nutrition
o Administer stool softeners as prescribed
o Administer daily rectal irrigation with normal saline to promote adequate elimination and prevent
obstruction as prescribed.

INTUSSUSCEPTION

 Telescoping of one portion of the bowel into another portion

 The condition results in obstruction to the passage of intestinal contents.

ASSESSMENT FINDINGS:
 Colicky abdominal pain that causes the child to scream and draw the knees to the
abdomen, similar to the fetal position.
  Vomiting of gastric contents
 Currant jelly-like stools containing blood and mucus
 Hypoactive and hyperactive bowel sounds
 Tender distended abdomen
 Palpable sausage-shaped mass in the upper right quadrant

INTERVENTIONS:
 Monitor for signs of perforation and shock as evidenced by fever, increased heart rate, changes in
blood pressure and respiratory distress.
 Decompression via nasogastric tube.
 Monitor for the passage of normal, brown stool, which indicates that the intussusception has
reduced itself.
 Prepare for shock to occur if no signs of hydrostatic reduction

Hydrostatic Reduction, air or fluid is used to exert pressure on area involved to lessen,
diminish, or rid the intestine of prolapse.
 Posthydrostatic reduction:
Monitor for the return of normal bowel sounds, passage of barium and characteristics of
stool.
Administer clear fluids, and advance the diet gradually.