Pediatric disorders

Pediatric Disorders:
Module
THE ILL AND HOSPITALIZED CHILD

STRESSORS and FEARS of HOSPITALIZATION
1. Separation Stages
2. Loss of Control
3. Body Injury
4. Pain
5. Immobility
6. Punishment and Rejection
PREPARATION for HOSPITALIZATION
1. Parents eagerly seek guidance from nurses on what and how much to tell
their children about an anticipated admission. The preparation a parent
makes for a child obviously varies according to the child's age and individual
experience. No matter what the child's age, however, parents should be
encouraged to convey a positive attitude.
2. Children between 2 and 7 years of age should be told about a scheduled
ambulatory or inpatient hospitalization as many days before the procedure as
the child's age in years.
3. On the day of admission, it is important for you to discuss the preparation the
child has received to ensure that the child and family accurately understand
the child's condition and upcoming procedures.
ASSESSMENT ON ADMISSION
1. Assess each child's level of preparation for a hospitalization on admission to
the facility. Be aware of not only what the child describes orally but also what
facial expressions or nervous manifestations may be indicating.
2. Interview parents on hospital admission for a nursing history to obtain the
information needed to plan nursing care.
3. Make a note of any medication or food allergy on the child's plan of care
4. Take and record the child's temperature, pulse, and respirations. Measure
height and weight to determine overall growth and to allow for determination
of surface area, the measurement on which medication dosage is calculated.
PAIN ASSESSMENT
For children, pain is not only a hurting sensation, but it can also be a confusing
one because a child did not anticipate the pain, cannot explain its presence, and
cannot always understand its cause. Because children may have difficulty
describing pain in a manner adults can understand, it is difficult to assess the
extent of their discomfort. Both helping children describe the type and extent of
pain they are feeling and performing active interventions to relieve pain are
important nursing roles.
METHODS OF PAIN ASSESSMENT

Pain Experience Inventory
- A tool consisting of eight questions for children and eight questions for the
child's parents. It is designed to elicit the terms a child uses to denote pain
and what actions the child thinks will best alleviate the pain.
Cries Neonatal Postoperative Pain Measurement Scale
- 10-point scale on which five physiologic and behavioral variables
frequently associated with neonatal pain can be assessed and rated:
 Amount and type of crying
 Need for oxygen administration
 Increased vital signs
 Facial expression
 Sleeplessness
Comfort Behavior Scale
- A pain rating scale devised by nurses to rate pain in very young infants.
On the first part of the scale, six different categories (alertness,
calmness/agitation, crying, physical movement, muscle tone, and facial
expression) are rated from 1 to 5. Six is the lowest score (no pain), and 30
is the highest (a great deal of pain). In addition to rating physical
parameters, nurses then observe the infant for 2 minutes and rate their
evaluation of the baby's pain on an analogue (1-to-10) visual scale.
FLACC Pain Assessment
- A scale by which health care providers can rate a child's pain when a child
cannot give input, such as during circumcision. It incorporates five types of
behaviors that can be used to rate pain: facial expression, leg movement,
activity, cry.
FACES Pain Rating Scale
- This scale consists of six cartoon-like faces ranging from smiling to tearful.
Explain to the child that each face from left to right corresponds to a
person who has no hurt up to a lot of hurt the words under each face to
describe the amount of pain the face represents.
Numerical or Visual Analog Scale
- It uses a line with end points marked “0 = no pain” on the left and “10 =
worst pain” on the right. Divisions along the line are marked in units from 1
to 9. Explain to children that the left end of the line (the 0) means a person
feels no pain. At the other end is a 10, which means a person feels the
worst pain possible. The numbers 1 to 9 in the middle are for “a little pain”
to “a lot of pain.” Ask children to choose a number that best describes their
pain.
Adolescent Pediatric Pain Tool

- It combines a visual activity and a numerical scale. On one half of the form
is an outline figure showing the anterior and posterior view of a child. To
use the tool, a child is asked to color in the figure drawing where he or she
feels pain. In addition, on the right side of the form, the child rates the pain
in reference to “no pain,” “little pain,” “medium pain,” “large pain,” and
“worst possible pain.” For a third activity, children are asked to point to or
circle as many words as possible on the form that describe their pain
(words such as horrible, pounding, cutting, and stinging)
- This is a useful tool for involving parents to talk with their child about his or
her pain. Reading the words together helps the child examine the type,
location, and level of pain he or she is experiencing. It also helps parents
to better understand what their child is experiencing.
PAIN MANAGEMENT
Non-Pharmacologic
1. Distraction - It aims at shifting a child's focus from pain to another activity or
interest.
2. Substitution of meaning - is a distraction technique to help a child place
another on a painful procedure. Children are often more adept at imagery
than adults because their imagination is less inhibited
3. Thought Stopping - a technique in which children are taught to stop anxious
thoughts by substituting a positive or relaxing thought. As with imagery, this
technique requires a great deal of practice before it is used in a painful
situation. For this technique, help the child to think of a set of positive things
about the approaching feared procedure.
4. Hypnosis - is not a common pain management technique with children but
can be very effective when a child is properly trained in the technique.
5. Magnet Therapy - is based on the belief that magnets can control or shift
body energy lines to restore health or relieve pain.
6. Music Therapy –the use of music for calming or improving well-being and can
be effective.
7. Yoga and Meditation - It offers a significant variety of proven health benefits,
such as increasing the efficiency of the heart, slowing the respiratory rate,
improving fitness, lowering blood pressure, promoting relaxation, reducing
stress, and allaying anxiety.
8. Acupuncture - involves the insertion of needles into critical positions in the
body to achieve pain relief. Although acupuncture is almost painless, children
can be very afraid of it at first because of the sight of the needles.
9. Transcutaneous Electrical Nerve Stimulation - involves applying small
electrodes to the dermatomes that supply the body portion where pain is
experienced.
Pharmacologic

1. Topical Anesthetic Cream - to reduce the pain of procedures such as
venipuncture, lumbar puncture, and bone marrow aspiration, a local
anesthetic cream or a solution of lidocaine and epinephrine is available
2. Oral Anlgesia – relatively easy to administer
3. Intramuscular Injection
4. Intravenous Administration - most rapid-acting route and the method of choice
in emergency situations.
5. Conscious Sedation - refers to a state of depressed consciousness usually
obtained through IV analgesia therapy. The technique allows a child to be
both pain-free and sedated for a procedure. Unlike with the use of general
anesthesia, protective reflexes are left intact and a child can respond to
instructions during the procedure.
6. Intranasal Administration
7. Local Anesthesia Injection
8. Epidural Analgesia - injection of an analgesic agent into the epidural space
just outside the spinal canal, it can be used to provide analgesia to the lower
body for 12 to 24 hours.
LEADING CAUSES of accidents/ injuries in children
Infancy:
 Aspiration
 Suffocation
 Fall
Toddler:
 Fall
 Drowning
 Poisoning
 Burn
Pre-schooler
 Drowning
 Motor accident
 Burn

DEVIATIONS FROM NORMAL IN THE NEWBORN
Pre-term or Low Birth Weight Infant
BACKGROUND OF THE STUDY
A preterm infant is usually defined as a live-born infant born before the end
of week 37 of gestation; another criterion used is a weight of less than 2,500
g (5 lb 8 oz) at birth. About 7% of all pregnancies end in preterm birth, and all
such infants need neonatal intensive care from the moment of birth to give
them their best chance of survival without neurologic after-effects (Petrou,
2003).
When a preterm infant is recognized by a gestational age assessment, watch
for the specific problems of prematurity, such as respiratory distress
syndrome, hypoglycemia, and intracranial hemorrhage.
Differences Between Small-for-Gestational-Age and Preterm Infants
Characteristic Small-for-Gestational-Age Infant Preterm Infant
Gestational age 24–44 wk Younger than 37 wk
Birthweight Under 10th percentile Normal for age
Congenital
malformations
Strong possibility Possibility
Pulmonary problems Meconium aspiration, pulmonary
hemorrhage, pneumothorax
Respiratory distress
syndrome
Hyperbilirubinemia Possibility Very strong possibility
Hypoglycemia Very strong possibility Possibility
Intracranial
hemorrhage
Strong possibility Possibility
Apnea episodes Possibility Very strong possibility
Feeding problems Most likely due to accompanying
problem such as hypoglycemia
Small stomach capacity;
immature sucking reflex
Weight gain in
nursery
Rapid Slow
Future restricted
growth
Possibly always be under 10th
percentile due to poor organ
development
Not likely to be restricted in
growth as “catch-up” growth
occurs

NEWBORN PRIORITIES IN FIRST DAYS OF LIFE
All newborns have eight priority needs in the first few days of life:
W - aste elimination establishment
A -dequate nourishment
T-emperature Control
E - xtrauterine circulation establishment
R- espiration initiation and maintenance
I- nfection prevention
D - evelopmental care / care that balances physiologic needs and stimulation for
best development
E - stablishment of an infant–parent relationship
These are also the priority needs of high-risk newborns. Because of small size or
immaturity or illness, fulfilling these needs, however, may require special
equipment or care measures. Not all newborns will be able to achieve full
wellness because of extreme insults to their health at birth or difficulty adjusting
to extrauterine life.
ETIOLOGY
- The exact cause of premature labor and early birth is rarely known.
- There is a high correlation between low socioeconomic level and early
termination of pregnancy.
- The major influencing factor in these instances appears to be inadequate
nutrition before and during pregnancy, as a result of either lack of money
for or lack of knowledge about good nutrition.

Factors Associated with Preterm Birth
- Low socioeconomic level
- Poor nutritional status
- Lack of prenatal care
- Multiple pregnancy
- Previous early birth
- Race (nonwhites have a higher
incidence of prematurity than whites)
- Cigarette smoking
- Age of the mother (highest incidence
is in mothers younger than age 20)
- Order of birth (early termination is
highest in first pregnancies and in
those beyond the fourth pregnancy)
- Closely spaced pregnancies
- Abnormalities of the mother's
reproductive system, such as
intrauterine septum
- Infections (especially urinary tract
infection)
- Obstetric complications, such as
premature rupture of membranes or
premature separation of the placenta
- Early induction of labor
- Elective cesarean birt

ASSESSMENT:
- Preterm infant appears small and underdeveloped.
- Head is disproportionately large (3 cm ormore greater than chest size).
- Skin is generally unusually ruddy because the infant has little
subcutaneous fat beneath it; veins are easily noticeable, and a high
degree of acrocyanosis may be present.
- The preterm neonate, 24 to 36 weeks, typically is covered with
vernixcaseosa. However, in very preterm newborns (less than 25 weeks'
gestation), vernix is absent because it is not formed this early in
pregnancy.
- Lanugo is usually extensive, covering the back, forearms, forehead, and
sides of the face, because this amount is present until late in pregnancy.
- Both anterior and posterior fontanelles are small.
- There are few or no creases on the soles of the feet.
- The eyes of most preterm infants appear small.
- Although difficult to elicit, pupillary reaction is present.
- A preterm infant has varying degrees of myopia (near-sightedness)
because of lack of eye globe depth.
- The cartilage of the ear is immature and allows the pinna to fall forward.
The ears appear large in relation to the head.
- If tested, reflexes such as sucking and swallowing will be absent if an
infant's age is below 33 weeks; deep tendon reflexes such as the achilles
tendon reflex are also markedly diminished.
- During an examination, a preterm infant is much less active than a mature
infant and rarely cries.
- If the infant does cry, the cry is weak and high-pitched.

POTENTIAL COMPLICATIONS
Because of immaturity, preterm infants are prone to a number of specific
conditions.
1. Anemia of Prematurity - Many preterm infants develop a normochromic,
normocytic anemia (normal cells, just few in number). The reticulocyte
count is low because the bone marrow does not increase its production
until approximately 32 weeks.
2. Kernicterus - a destruction of brain cells by invasion of indirect bilirubin.
Preterm infants are more prone to the condition than term infants because
with the acidosis that occurs from poor respiratory exchange, brain cells
are more susceptible to the effect of indirect bilirubin than normally.
3. Persistent Patent DuctusArteriosus - Because preterm infants lack
surfactant, their lungs are noncompliant, so it is more difficult for them to
move blood from the pulmonary artery into the lungs. This condition leads
to pulmonary artery hypertension, which may interfere with closure of the
ductusarteriosus.
4. Periventricular/Intraventricular Hemorrhage - Preterm infants are prone
to periventricular hemorrhage (bleeding into the tissue surrounding the
ventricles) or intraventricular hemorrhage (bleeding into the ventricles);
these conditions occur in as many as 50% of infants of very low
birthweight. This occurs because preterm infants have both fragile
capillaries and immature cerebral vascular development.
NURSING DIAGNOSIS
Because a preterm infant has few body resources, both physiologic and
psychological stress must be reduced as much as possible and interventions
initiated gently to prevent depletion of resources. Close observation and analysis
of findings are essential to managing problems quickly.
1. Nursing Diagnosis: Impaired gas exchange related to immature
pulmonary functioning

Outcome Evaluation:
Many preterm babies, particularly those under 32 weeks of age,
have an irregular respiratory pattern (a few quick breaths, a period of 5 to
10 seconds without respiratory effort, a few quick breaths again, and so
on).
There is no bradycardia with this irregular pattern (sometimes
termed periodic respirations).
2. Nursing Diagnosis: Risk for imbalanced nutrition, less than body
requirements related to additional nutrients needed for maintenance of
rapid growth, possible sucking difficulty, and small stomach.
Outcome Evaluation:
Infant's weight follows percentile growth curve; skin turgor is good;
specific gravity of urine is maintained between 1.003 and 1.030; infant has
no more than 15% weight loss in first 3 days of life and continues to gain
weight after this point.
3. Nursing Diagnosis: Risk for infection related to immature immune
defenses in preterm infant
Outcome Evaluation
Temperature is maintained at 97.6°F (36.5°C) axillary; further signs
and symptoms of infection such as poor growth or a reduced temperature
are absent

CONGENITAL HEART DEFECTS
Congenital heart disease refers to a problem with the heart's structure and
function due to abnormal heart development before birth. Congenital means
present at birth.
ACYANOTIC HEART DISEASE
Acyanotic heart disease is a broad term for any congenital heart defect in
which all of the blood returning to the right side of the heart (shunt that moves
blood from the arterial to the venous system or left-to-right shunts), passes
through the lungs and pulmonary vasculature in the normal fashion. The common
forms of acyanotic congenital heart defects are those where there is a defect in
one of the walls separating the chambers of the heart, or obstruction to one valve
or artery.
TYPES OF ACYANOTIC HEART DISEASE
1.) ATRIAL SEPTAL DEFECT
DEFINITION
-Is a form of congenital heart defect that enables blood flow between the left and
right atria via the interatrial septum.
-The interatrial septum is the tissue that divides the right and left atria. Without
this septum, or if there is a defect in this septum, it is possible for blood to travel
from the left side of the heart to the right side of the heart, or vice versa.

-This results in the mixing of arterial and venous blood, which may or may not be
clinically significant.
ETIOLOGY
The heart is forming during the first 8 weeks of fetal development. It
begins as a hollow tube, then partitions within the tube develop that eventually
become the septa (or walls) dividing the right side of the heart from the left. Atrial
septal defects occur when the partitioning process does not occur completely,
leaving an opening in the atrial septum.
Some congenital heart defects may have a genetic link, either occurring due to a
defect in a gene, a chromosome abnormality, or environmental exposure,
causing heart problems to occur more often in certain families. Most atrial septal
defects occur sporadically (by chance), with no clear reason for their
development.
SIGNS AND SYMPTOMS
 Child tires easily when
playing.
 Fatigue.
 Sweating.
 Rapid breathing.
 Shortness of breath.
 Poor growth.
DIAGNOSIS
 Physical exam auscultation of the heart- there is a loud harsh systolic
murmur in the left sternal border at the 3rd-4th interspaces
 Echocardiography- an atrial septal defect may be seen on color flow
imaging as a jet of blood from the left atrium to the right atrium.
 Transcranial Doppler (TCD) Bubble study- This method reveals the
cerebral impact of the ASD or PFO.
 Electrocardiogram- Individuals with atrial septal defects may have a
prolonged PR interval (a first degree heart block).
 Chest x-ray - a diagnostic test which uses invisible electromagnetic energy
beams to produce images of internal tissues, bones, and organs onto film.
With an ASD, the heart may be enlarged because the right atrium and
ventricle have to handle larger amounts of blood flow than normal.
NURSING DIAGNOSIS
 Activity intolerance
 Decreased cardiac output
 Deficient knowledge
(diagnosis and treatment)
 Fatigue
 Impaired gas exchange
 Risk for infection
EXPECTED OUTCOMES FOR NURSING CARE PLAN
 The patient will carry out activities of daily living without weakness or
fatigue.
 The patient will maintain hemodynamic stability, and cardiac output will
remain adequate.

 The patient or her parents will verbalize understanding of the atrial septal
defect and plans for treatment.
 The patient will report that she has more energy.
 The patient will maintain adequate ventilation and oxygenation.
 The patient will remain free from signs and symptoms of infection.
TREATMENT/MANAGEMENT
 Surgery- to close the defect for children 1-3 years of age. This is to
prevent risk for infectious endocaditis and eventual heart failure.
 Cardiac catheterization- technique if the defect is small wherein the edge
of the opening of the septum is sutured.
 Open heart surgery and cardiopulmonary bypass- for large defects.
POST-OPERATIVE CARE
 Ventilator - a machine that helps your child breathe while he/she is under
anesthesia during the operation.
 Intravenous (IV) catheters - small, plastic tubes inserted through the skin
into blood vessels to provide IV fluids and important medications that help
your child recover from the operation.
 Arterial Nasogastric (NG) tube - a small, flexible tube that keeps the
stomach drained of acid and gas bubbles that may build up during
surgery.
 Heart monitor - a machine that constantly displays a picture of your
child's heart rhythm, and monitors heart rate, arterial blood pressure, and
other values.
 Closely monitor vital signs, central venous and intra-arterial pressures,
and intake and output.
 Watch for atrial arrhythmias.
 Give an antibiotic and an analgesic, as ordered.
 Provide range-of-motion exercises and coughing and deep-breathing
exercises.
PROGNOSIS
With a small to moderate atrial septal defect, a person may live a normal
life span without symptoms. Larger defects may cause disability by middle age
because of increased blood flow and shunting of blood back into the pulmonary
circulation. Some patients with ASD may have other congenital heart conditions,
such as a leaky valve.

2.) VENTRICULAR SEPTAL DEFECT
DEFINITION
A ventricular septal defect is an abnormal opening in the wall (septum)
that divides the two lower chambers of the heart (ventricles). A Ventricular septal
defect closure is a procedure performed to correct this defect.
ETIOLOGY
The cause of VSD (ventricular septal defect) includes the incomplete
looping of the heart during days 24-28 of development. Faults with NKX2.5 gene
can cause this.
Congenital VSDs are frequently associated with other congenital conditions, such
as Down syndrome
DIAGNOSIS
Cardiac auscultation- VSD causes a pathognomonic holo- or pansystolic
murmur. Auscultation is generally considered sufficient for detecting a significant
VSD.
Ultrasound (echocardiography)
CLINICAL MANIFESTATION
 Tachypnea is typically the first presenting symptom.
 Dyspnea results in poor nursing and frequent rest during feedings
 Hepatomegaly may be present.
 The murmur of VSD is due to left-to-right shunting at the ventricular level.
Small ventricular septal defects are typically louder than larger ones. The
murmur of a VSD is heard best at the left lower sternal border.
 Right-to-left shunting at the VSD are not audible due to a small amount of
pressure difference between the right and left ventricles.
 A loud third heart sound or diastolic rumble is heard with large left-to-right
shunting due to increased flow across the mitral valve.
 A thrill is felt in many cases, particularly beyond infancy.

NURSING DIAGNOSIS
 Alteration in tissue perfusion
 Risk for infection
 Fatigue
 Weakness.
 Ineffective breathing pattern
SIGNS AND SYMPTOMS
 Pansystolic (Holosystolic)
murmur (depending upon the
size of the defect)
 +/- Palpable thrill (palpable
turbulence of blood flow).
 Heart sounds are normal.
 sweaty and tachypnoiec
(breathe faster) with feeds
 easy fatigue
 If the opening is SMALL- 85% closes spontaneously.
 MODERATE- cardiac catheterization, Cardiopulmonary bypass, the edges
of the septal opening is sutured.
 LARGE- (over 3 mm) open heart surgery, Silastic or Dacron patch is
sutured to occlude the space.
PRE-OPERATIVE TEACHING:
 If at all possible, it is important that the patient be free of infection prior to
going to surgery.
 If the patient is due for immunizations within a week of surgery, contact
the Congenital Heart Surgery Clinic and ask to speak to the clinic nurse
 Patients undergoing cardiac surgery frequently need blood products
 Provide emotional support to the family.
 Signing of consent.
POST-OPERATIVE TEACHING
 Watch out for the following: redness, swelling, or oozing/bleeding from
incision, fever, altered mental status, excessive fatigue, feeding/eating
problems, prolonged or worsening pain
 Avoid activities or movement for 4-6 weeks.
PROGNOSIS
This is excellent for most patients. The vast majority are able to live a
normal and unrestricted life. Re-operations for residual VSDs are now
uncommon.

3.) PATENT DUCTUS ARTERIOSUS
DEFINITION
Is a congenital disorder in heart wherein a neonate's ductus arteriosus
fails to close after birth. The condition leads to abnormal blood flow between the
aorta and pulmonary artery, two major blood vessels surrounding the heart.
The ductus arteriosus (DA) is the vascular connection between the pulmonary
artery and the aortic arch.
ETIOLOGY
Before birth, the ductus arteriosus allows blood to bypass the baby's lungs
by connecting the pulmonary arteries (which supply blood to the lungs) with the
aorta (which supplies blood to the body). Soon after the infant is born and the
lungs fill with air, this blood vessel is no longer needed. It will usually close within
a couple of days. If the ductus arteriosus does not close, there will be abnormal
blood circulation between the heart and lungs.
RISK FACTOR
PDA is rare. It affects girls more often than boys. The condition is more
common in premature infants and those with neonatal respiratory distress
syndrome. Infants with genetic disorders, such as Down syndrome, and whose
mothers had German measles (rubella) during pregnancy are at higher risk for
PDA.
SIGNS AND SYMPTOMS
 Bounding pulse
 Fast breathing
 Poor feeding habits
 Shortness of breath
 Sweating while feeding
 Tiring very easily
 Poor growth

 Wide pulse pressure
 Murmur can be heard in upper left sternal border or under the left clavicle
(older children)
 Short grade II and III harsh systolic sound (newborn)
 Ventricle enlargement
DIAGNOSIS
 AUSCULTATION- murmur
 Echocardiogram. An echocardiogram uses sound waves to produce a
video image of the heart. This image can help doctors see the heart
chambers and evaluate how well the heart is pumping. This test also
checks the heart valves and looks for any other heart defects.
 Chest X-ray. An X-ray image helps the doctor see the condition of your
baby's heart and lungs and the amount of blood in the lungs
 Electrocardiogram (ECG). This test records the electrical activity of the
heart. This test helps diagnose heart defects or rhythm problems.
 Cardiac catheterization. This test isn't usually necessary for diagnosing a
PDA alone, but may be done to examine other congenital heart defects
found during an echocardiogram
 Cardiac computerized tomography (CT) or magnetic resonance imaging
(MRI).
TREATMENT
 PD is open because of stimulation of prostaglandins (PGE1) from the
placenta and decrease O2 of fetal blood. If PGE1 decreases and O2
increases PD is stimulated to close.
 If PD doesn’t close spontaneously IV INDOMETHACIN and ibuprofen,
prostaglandin inhibitors are given.Side effects: Decrease glomelular
filtration, impaired platelet aggregation, and diminished G.I. and Cerebral
blood flow.
 Dacron-coated staimless steel coils by interventional cardiac
catheterization (6 mos-1 yr).
 LARGE DUCTAL LIGATION
 TRANSCATHETER DEVICE CLOSURE

(Cardiomegaly and Pulmonary edema). 22 days of life, before the surgery.
Following surgical ligation of PDA, there is improving edema and less
cardiomegaly.
PROGNOSIS
Adults and children can survive with a small opening remaining in the
ductus arteriosus. Treatment, including surgery, of a larger PDA is usually
successful and frequently occurs without complications. Proper treatment allows
children and adults to lead normal lives.

4.) COARCTATION OF AORTA
DEFINITION
Is a narrowing of the aorta, the large blood vessel that branches off your
heart and delivers oxygen-rich blood to your body. When this occurs, your heart
must pump harder to force blood through the narrow part of your aorta.
Coarctation of the aorta usually occurs beyond the blood vessels that branch off
to your upper body and before the blood vessels that lead to your lower body.
This often means you'll have high blood pressure in your arms, but low blood
pressure in your legs and ankles.
ETIOLOGY AND RISK FACTOR
The aorta carries blood from the heart to the vessels that supply the body
with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to
pass through the artery.
Aortic coarctation is more common in persons with certain genetic disorders,
such as Turner syndrome. However, it can also be due to birth defects of the
aortic valves.
Aortic coarctation is one of the more common heart conditions that are present at
birth (congenital heart conditions). It is usually diagnosed in children or adults
under age 40.
SIGNS AND SYMPTOMS
BABIES WITH SEVERE COARCTATION
 Pale skin
 Irritability
 Heavy sweating
 Difficulty breathing
OLDER CHILDREN
 High blood pressure  Shortness of breath,
especially during exercise

 Headache
 Muscle weakness
 Leg cramps or cold feet
 Nosebleeds
DIAGNOSIS/DIAGNOSTIC PROCEDURE
 HISTORY and PHYSICAL
ASSESSMENT
 The pulse in the femoral
(groin) area or feet will be
weaker than the pulse in the
arms or the carotid (neck).
Sometimes, the femoral pulse
may not be felt at all.
 The blood pressure in your
legs is usually weaker than in
the arms. Blood pressure is
usually higher in the arms
after infancy. BP in arms is
20mmhg higher than in the
leg
 Echocardiography
 ECG-show that you might
have a thickened heart
muscle (ventricular
hypertrophy).
 X-ray may show an enlarged
heart or a narrowing in the
aorta at the site of the
coarctation. Left-sided heart
enlargement.
 MRI- reveals the location of
the coarctation of the aorta.
 MRI or MR angiography of the
chest may be needed in older
children
ASSESSMENT
 Slight Coarctation- absent of palpable femoral pulse
 Obstruction is proximal- absent of brachial pulse
 As infant grow older- leg pain on exertion due to diminish blood supply to
lower extremities
 Surgical resection of the
narrow segment if there is
arterial hypertension.
 angioplasty
 Cardiac catheterization and
aortography
 DACRON graft
 Balloon Angioplasty
NURSING DIAGNOSIS
 Fatigue
 Activity intolerance
 Ineffective breathing pattern
secondary to pulmonary
hypertension
 sleep deprivation secondary
to discomfort and irritability
PROGNOSIS
Coarctation of the aorta can be cured with surgery. Symptoms quickly get
better after surgery. However, there is an increased risk for death due to heart
problems among those who have had their aorta repaired. Without treatment,
most people die before age 40. For this reason, doctors usually recommend that
the patient has surgery before age 10. Most of the time, surgery to fix the
coarctation is done during infancy. Narrowing or coarctation of the artery can

return after surgery. This is more likely in persons who had surgery as a
newborn.
CYANOTIC HEART DEFECT
Definition:
 Blood is shunted from the venous to the arterial system as a result of
abnormal communication between the two
 Deoxygenated blood to oxygenated blood
 Right to left shunt
Types:
 Transposition of great artery
 Tetralogy of fallot
TRANSPOSITION OF GREAT ARTERY
Definition:
 Defect with mixed blood flow
 Aorta arises from right ventricle instead of left
 Pulmonary artery arises from left ventricle instead of right
 Atrial and ventricular septal defects occur in connection with transposition
making the entire heart one mixed circulatory system
 Large newborn(9-10lbs), more on boys
 5% of congenital anomalies
Etiology/Pathophysiology:
The pulmonary and systemic circulations function in parallel, rather than in
series. Oxygenated pulmonary venous blood returns to the left atrium and left
ventricle but is re-circulated to the pulmonary vascular bed via the abnormal
pulmonary arterial connection to the left ventricle. Deoxygenated systemic
venous blood returns to the right atrium and right ventricle where it is
subsequently pumped to the systemic circulation, effectively bypassing the lungs.
This parallel circulatory arrangement results in a deficient oxygen supply to the
tissues and an excessive right and left ventricular workload. It is incompatible
with prolonged survival unless mixing of oxygenated and deoxygenated blood
occurs at some anatomic level.

Signs and Symptoms:
 Cyanotic from birth
 No murmurs/ various murmurs
Laboratory/Diagnostic Findings:
 Echocardiography reveals enlarged heart
 ECG may or may not reveal enlarged heart
 Decreased oxygen saturation in cardiac catetherization
Management:
 Patent ductus arteriosus
 PGE administration
 Balloon atrial septal pull through operation
 Cardiac catheterization
 Arterial switch procedure (1week to 3 mos, 95%)
Nursing diagnosis:
1. Ineffective cardiopulmonary tissue perfusion related to impaired cardiac
function and increased cardiac workload.
2. Excess fluid volume related to impaired cardiac contractility and venous
congestion
3. Activity intolerance related to effects of heart failure.
4. Risks for injury related to congenital heart defect and surgery.
5. Risks for infection related to immature immune system and neonatal age.
6. Risks for imbalanced nutrition, less than body requirements.
Implementation
1. Administer supplemental oxygen.
2. Elevate head of the bed 30 to 60 degrees or have child sit upright.
3. Assess vital signs including heart rate, pulse and respirations.
Auscultate heart and lung sounds.
4. Obtain baseline weight and monitor at least daily.
5. Monitor intake and output and urine specific gravity.
6. Provide a balance of activity and rest periods.
7. Provide small and frequent meals.
8. Assesses infant's skin integrity; sensory impairments, and immune
status.
9. Implements protective measures to prevent injury caused by electrical,
thermal, chemical, or physical sources, including

* verifying allergies,
* applying safety devices,
* ensuring prep solution does not run under
electrosurgical grounding pad,
* performing required counts, and
* using supplies and equipment within safe parameters.
Prognosis:
The child's symptoms will improve after surgery to correct the defect. Most
infants who undergo arterial switch do not have symptoms after surgery and live
normal lives. If corrective surgery is not performed, the life expectancy is months.
TETRALOGY OF FALLOT
Definition:
 Defect with decreased pulmonary blood flow
 10% of children with congenital disease
 Four anomalies: pulmonary stenosis
VSD
Dextraposition of aorta
Hypertrophy of right ventricle
 15% of children with this disorder show deletion abnormality of
chromosome22
 Pulmonary stenosis
A narrowing of the right ventricular outflow tract and can occur at the pulmonary
valve (valvular stenosis) or just below the pulmonary valve (infundibular
stenosis). Infundibular pulmonic stenosis is mostly caused by overgrowth of the
heart muscle wall (hypertrophy of the septoparietal trabeculae), however the
events leading to the formation of the overriding aorta are also believed to be a
cause. The pulmonic stenosis is the major cause of the malformations, with the
other associated malformations acting as compensatory mechanisms to the
pulmonic stenosis. The degree of stenosis varies between individuals with TOF,
and is the primary determinant of symptoms and severity. This malformation is
infrequently described as sub-pulmonary stenosis or subpulmonary obstruction.

 Overriding aorta
An aortic valve with biventricular connection, that is, it is situated above the
ventricular septal defect and connected to both the right and the left ventricle.
The degree to which the aorta is attached to the right ventricle is referred to as its
degree of "override." The aortic root can be displaced toward the front (anteriorly)
or directly above the septal defect, but it is always abnormally located to the right
of the root of the pulmonary artery. The degree of override is quite variable, with
5-95% of the valve being connected to the right ventricle.
 Right ventricle hypertrophy
The right ventricle is more muscular than normal, causing a characteristic boot-
shaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the
misarrangement of the external ventricular septum, the right ventricular wall
increases in size to deal with the increased obstruction to the right outflow tract.
This feature is now generally agreed to be a secondary anomaly, as the level of
hypertrophy generally increases with age.
 Ventricular septal defect
A hole between the two bottom chambers (ventricles) of the heart. The defect is
centered around the most superior aspect of the ventricular septum (the outlet
septum), and in the majority of cases is single and large. In some cases
thickening of the septum (septal hypertrophy) can narrow the margins of the
defect.
Signs and Symptoms:
 May not exhibit high degree of cyanosis immediately
 Bluish tint
 Polycythemia
 Severe dyspnea
 Growth restriction
 Clubbing of fingers
 Fainting
 Hypoxic episode (tet spell)
 Cognitive challenge
 Increased Hg, Hct
 Echocardiography shows enlarged chamber of right heart

 Echocardiography shows decrease size in pulmonary artery and reduced
blood flow through lungs.
 ECG shows enlarged chamber of right heart
 Cardiac catheterization and angiography evaluate extent of defect
 Polycythemia
 Reduced oxygen saturation
Management:
 Propranolol
 Administer oxygen
 Knee chest position
 Blalock taussig
 Brock procedure
Nursing diagnosis:
1. Decreased cardiac out put related to structural defect.
2. Activity intolerance related to imbalance between oxygen supply and
demand.
3. Altered growth and development related to inadequate oxygen, nutrients
to tissue and social isolation.
4. High risk for infection related to debilitated physical status.
5. Altered family process related to having a child with a heart condition.
6. High risk for injury (complications) related to cardiac condition and
therapies.
Implementation
1. Administer supplemental oxygen.
2. Elevate head of the bed 30 to 60 degrees or have child sit upright.
3. Assess vital signs including heart rate, pulse and respirations.
Auscultate heart and lung sounds.
4. Allow time for frequent of rest.
5. Help child to select activities appropriate to age, condition and
capabilities.
6. Avoid extremes of environmental temperature.
7. Provide well balanced highly nutritive diet.
8. Avoid contact with infected persons.
9. Discuss with parents their fears regarding child symptoms.
10.Encourage family to participate in care of child while hospitalized.
11.Encourage family to include others in child’s care to prevent their own
exhaustion.

Prognosis:
Most cases can be corrected with surgery. Babies who have surgery usually do
well. Ninety percent survive to adulthood and live active, healthy, and productive
lives. Without surgery, death usually occurs by the time the person reaches age
20.
Patients who have continued, severe leakiness of the pulmonary valve may need
to have the valve replaced.
Regular follow-up with a cardiologist to monitor for life-threatening arrhythmias
(irregular heart rhythms) is recommended
TRANSPOSITION OF GREAT ARTERIES

Acute Rheumatic Fever
Background of Case
- Autoimmune disease that occurs as a reaction to a group A beta-
hemolytic streptococcal infection
- Follows attack of pharyngitis, tonsillitis, scarlet fever, “strep throat”, or
impetigo
- Inflammation from immune system will lead to fibrin deposits on
endocardium and valves , and body joints
Etiology
- group A beta-hemolytic streptococcal infection (GABS)
Laboratory findings
1. High ESR
2. Anemia, leucocytosis
3. Elevated C-reactive protein
4. ASO titre >200 Todd units.(Peak value attained at 3 weeks,then comes
down to normal by 6 weeks)
5. Anti-DNAse B test
6. Throat culture-GABHstreptococci
7. ECG- prolonged PR interval, 2nd or 3rd degree blocks, ST-depression, T-
inversion
8. 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV
dilatation, pericardial effusion, decreased contractility
Signs and Symptoms
 Major:
1. Subcutaneous nodules
2. Pancarditis
3. Arthritis
4. Chorea
5. Erythema marginatum
 Minor
1. Fever
2. Arthralgia
3. Previous rheumatic fever attacks
4. All that is stated in the laboratory findings
(Using Jones Criteria)*the presence of 2 MAJOR criteria or of 1 MAJOR and 2
MINOR criteria indicates a high probability of acute rheumatic fever, if supported
by evidence of Group A streptococcal infection

NANDA Problems
1. Nursing Diagnosis: Risk for non-adherence to drug therapy related to
knowledge deficit about importance of long-term therapy
Outcome Evaluation: child takes oral penicillin daily; absence of symptoms
of throat infection; vital signs are within age-acceptable parameters.
2. Nursing Diagnosis: Situational low-esteem related to chorea movements
secondary to rheumatic fever
Outcome Evaluation: child expresses frustration with inability to control
movements; continues to feed and dress self with help as needed.
Children may have difficulty feeding themselves because of chorea. They
may be also be emotionally unstable and cry easily. Emphasize the
transitory nature of the chorea; stress that is frustrating to have to be fed
and to be unable to use your hands meaningfully, but that is lack of
coordination will pass without permanent effects. Provide toys and games
that do not require fine coordination, because it may be frustrating to try to
do something such as move checkers or chessmen on a board (a typical
low activity game). Children with chorea who are on bed rest may need to
have bedrails padded so they do not injure themselves with thrashing
movements.
Nursing and Medical Management
1. Bed Rest
2. Monitor vital signs
3. Penicillin Therapy/ single intramuscular injection of benzathine penicillin
4. Oral ibuprofen
5. Corticosteroids: SE- Hirsutism, Cushing’s Syndrome
6. Phenobarbital and Diazepam
Prognosis
- Rheumatic fever can recur whenever the individual experience new GABH
streptococcal infection, if not on prophylactic medicines
- Good prognosis for older age group & if no pancarditis during the initial
attack
- Bad prognosis for younger children & those with pancarditis with valvar
lesions

KAWASKI DISEASE (Mucocutaneous lymph node syndrome)
Definition:
 A febrile disease
 Multisystem disorder almost
exclusively in children before
the age of puberty.
 The peak incidence is in boys
under 4 years old
 The incidence is higher in late
winter and spring
 Unknown cause
 Develops in genetically
predisposed clients
 Unknown Etiology
 Pathophysiology
1. After the infection (upper
respiratory infection)
2. Altered immune function
occurs
3. Increase in the antibody
production
4. Creates circulating immune
complexes that bind
5. to the endothelium and
cause inflammation
6. The inflammation of the
blood vessels (Vasculitis)
leads to:
 Aneurysms
 Platelet accumulation
 Formation of Thrombi
 Obstruction in the heart
and blood vessels
Signs and Symptoms:
• High fever( 102 to 104⁰F [39
to 40⁰C]) Does Not Respond
toAntipyretic
• Child acts lethargic/irritable
• May have reddened and
swollen hands & feet
• Bulbar mucous membrane of
the eyes become inflamed
(Conjunctivitis)
• Strawberry tongue and red,
cracked lips
• Rashes occur(diaper area)
• Cervical lymph node become
enlarged
• Internal lymph nodes swell
• May develop abdominal pain,
anorexia and diarrhea
• Joints may swell and
redden(simulating arthritic
process)
 WBC and ESR are both elevated
Progression of the Disease:
10 days after onset(subacute phase)
• The skin desquamates (palms
and soles)
• Platelet count rises (increased
clotting necrosis of distal
body cells, particularly in the
fingertips)
• Aneurysms may form in
coronary arteries(CHA)
• Sudden death from
accumulating thrombi or
rupture of an aneurysm
(MOST DANGEROUS
PHASE)

25 days after onset (Convalescent Phase)
• Begins @ about the 25th day and last until 40 days
STAGE III last from 40 days until ESR returns to normal
Management:
 Administration of acetysalicylic acid or ibuprofen (dec I & PA)
 Abciximab – platelet receptor inhibitor specific for KD
 IV immune globulin can also be administed (reduce immune response)
 Coronary artery bypass surgery (CAD from stenosis of the Coronary
Arteries)
NOTE: *Steriods  CONTRAINDICATED (increase aneurysm formation)*
Nursing diagnosis:
1. Conjunctivitis: "L & R eye redness and yellow drainage" (Doesn't need
evidence. This IS the evidence)
2.Gingivitis: "Gum irritation M/B redness and swelling at upper and lower gums"
3.Rash: "Impaired Skin Integerity at bilateral hands M/B red rash with exfoliation"
4."Impaired Skin Integrity at bilateral feet M/B red rash with exfoliation"
5.Hand edema: "Impaired Tissue Integrity M/B 2+ pitting edema at bilateral
hands"
6.Foot edema: "Impaired Tissue Integrity M/B 3+ pitting edema at bilateral feet"
Joint inflammation: "Pain M/B warmth, redness and swelling at bilateral knees
and pt. states 6/10 pain on 0-10 P/S"
Implementation
Monitoring
1. Monitor pain level and child’s response to analgesics.
2. Institute continual cardiac monitoring and assessment for complications;
report arrhythmias.
o Take vital signs as directed by condition; report abnormalities.
o Assess for signs of myocarditis (tachycardia, gallop rhythm, chest
pain).
o Monitor for heart failure (dyspnea, nasal flaring, grunting,
retractions, cyanosis, orthopnea, crackles, moist respirations,
distended jugular veins, edema).
Closely monitor intake and output, and administer oral and I.V fluids as
ordered.
Monitor hydration staus by checking skin turgor, weight, urinary output,
specific gravity, and presence of tears.

Observe mouth and skin frequently for signs of infection.
Supportive care
1. Allow the child periods of uninterrupted rest. Offer pain medication
routinely rather than as needed during stage I. Avoid NSAIDS if the child
is in aspirin therapy.
2. Perform comfort measures related to the eyes.
o Conjunctivities can cause photosensitivity, so darken the room,
offer sunglasses.
o Apply cool compress.
o Discourage rubbing the eyes.
o Instill artificial tears to soothe conjunctiva.
3. Monitor temperature every 4 hours. Provide sponge bath if temperature
above normal.
4. Perform passive range of motion exercises every 4 hours while the child is
awake because movement may be restricted.
5. Provide quiet and peaceful environment with diversional activities.
6. Provide care measures for oral mucous membrane.
o Offer cool liquids like ice chips and ice pops.
o Use soft toothbrush only.
o Apply petroleum jelly to dried, cracked lips.
7. Provide skin measures to improve skin integrity.
o Avoid use of soap because it tends to dry skin and make it more
likely to breakdown.
o Elevate edematous extremities.
o Use smooth sheets.
o Apply emollients to skin as ordered.
o Protect peeling of skin, observe for signs of infection.
8. Offer clear liquids every hour when the child is awake.
9. Encourage the child to eat meals and snack with adequate protein.
10.Infuse I.V fluids through a volume control device if dehydration is present,
and check the site and amount hourly.
11.Explain all procedures to the child and family.
12.Encourage the parents and child to verbalize their concerns, fears, and
questions.
13.Practice relaxation techniques with child, such as relaxation breathing,
guided imagery, and distraction.
14.Prepare the child for cardiac surgery or thrombolytic therapy if
complications develop.
15.Keep the family informed about progress and reinforce stages and
prognosis.

Prognosis:
A large majority of children who develop Kawasaki disease recover within
two weeks and experience no long-lasting effects. As many as 25 percent of
sufferers may develop heart problems, but that percentage drops dramatically to
below 5 percent with quick treatment. Only about 1 percent of cases prove fatal,
but because that possibility exists, you must know what symptoms to watch for
and what steps to take to prevent immediate and future complications.

Infective Endocarditis
Endocarditis is inflammation and infection of the endocardium or valves of the
heart. It may occur in a child without heart disease but more commonly occurs as
a complication of congenital heart disease such as tetralogy of Fallot, VSD, or
coarctation of the aorta.
Bacteria or other infectious substance can enter the bloodstream during certain
medical procedures, including dental procedures, and travel to the heart, where it
can settle on damaged heart valves. The bacteria can grow and may form
infected clots that break off and travel to the brain, lungs, kidneys, or spleen.
Etiology: Streptococci of the viridans type
Pathophysiology: A high-velocity flow through a stenotic or incompetent valve
or an abnormal communication between systemic and pulmonary circulations
causes turbulence downstream from the opening. This turbulence damages or
denudes the endothelium, to which platelets and fibrin adhere, and a small,
sterile" nonbacterial thrombotic endocardial lesion" forms. In addition, indwelling
intravascular catheters in the right heart may directly traumatize the endocardium
or valvular endothelium. Circulating bacteria and inflammatory cells adhere to
and grow in these thrombi, forming infected vegetation. Infection may occur on
the wall, where the turbulent jet strikes, or downstream, near the orifice, where
the flow eddies. Once vegetation forms, the constant blood flow may result in
embolization to virtually any organ in the body. A brisk immunologic response is
produced.
Manifestation:
 Paleness
 Anorexia
 weight loss
 Chills
 Arthralgia
 Sweating at night
 murmur become audible
 petechiae in conjunctiva
 RUQ abdominal pain

Laboratory Test
 CBC count: Anemia is present in 70-90% of patients and is usually
normocytic and normochromic. Leukocytosis is noted in 20-30% of
patients.
 ESR and C-reactive protein level: The ESR is elevated in almost all
patients except for those with congestive heart failure (CHF), renal failure,
and disseminated intravascular coagulation (DIC). The mean ESR is 55
mm/h. The C-reactive protein, although nonspecific, is elevated in most
patients but decreases with successful treatment. Levels of C-reactive
protein may be used to monitor response to antibiotic therapy..
 Urinalysis may reveal proteinuria (50-60%) and/or microscopic hematuria
(30-50%).
Treatment
Prophylactic administration
antibiotic
Penicillinase-resistant
penicillin (Nafcillin) UNIPEN
via IV (cvc)
Nursing Diagnosis:
 Decreased cardiac
output related to
congenital structural
disorder
 Ineffective tissue
perfusion related to
inadequate cardiac
output
Prognosis:
The prognosis of bacterial endocarditis varies with the etiologic agent. Infection
by a penicillin-sensitiveStreptococcus, diagnosed early, has a cure rate of almost
100%. Because many infections are diagnosed late or due to resistant
organisms, the average mortality rate is approximately 20-25%.

UTI- Urethritis
Background:
Urethritis is inflammation of the urethra. The main symptom is dysuria, which is
painful or difficult urination.
Etiology:
>gonococcocal urethritis
Other causes include:
 Adenovirus
 Uropathogenic Escherichia
coli (UPEC)
 Herpes simplex
 Mycoplasma genitalium
 Reiter's syndrome
 Trichomonas spp.
 Ureaplasma urealyticum
Signs and symptoms/ Clinical Manifestations:
>In men- purulent discharge
>Frequency
>Dysuria
Labs and Diagnostics:
>Culture and sensitivity testing
Nursing Management:
>Removal of etiologic agent- by
administering doctor-prescribed
systemic and topical antibiotics
>Sitz bath
>Increases fluid intake
>Advise client to avoid coitus
NANDA problems:
Impaired Urinary elimination r/t irritation and inflammation of the urethral mucosa
Acute pain r/t irritation and inflammation of the urethral mucosa
Prognosis:
With the correct diagnosis and treatment, urethritis usually clears up without any
complications.
However, urethritis can lead to permanent damage to the urethra (scar tissue
called urethral stricture) and other urinary organs in both men and women.

UTI: Cystitis
Background:
Is a term that refers to urinary bladder inflammation that results from any one of a
number of distinct syndromes. It is most commonly caused by a bacterial
infection in which case it is referred to as a urinary tract infection
Etiology:
>gram negative bacteria (E. coli, Klebsiella, Enterobacter, Proteus)
>Candida spp.
>Chlamydia trachomitis, Trichomonas vaginalis, Neiseria gonorrhea
>change in voiding habits
>burning pain on urination (dysuria)
>frequency
>urgency
>voiding in small amounts
>incomplete bladder emptying
>hematuria
>urine culture
>dipstick test
Nursing Management:
>Inhibit bacterial growth- give adequate
instructions about antibiotics therapy and dietary
and activity restrictions.
>Advice patient to Modify Diet- dietary changes
needed to keep urine acidic and to reduce bladder
irritation by avoiding spicy foods, caffeinated and
alcoholic beverages
> Advice patient to Increase fluid intake- to flush
urinary system
>Prevent complications- educate client about inc manifestations that might result
from infection of the upper UT
NANDA problems:
Impaired Urinary elimination r/t irritation and inflammation of the bladder mucosa
Acute pain r/t irritation and inflammation of the bladder and mucosa
Prognosis:
The prognosis for recovery from uncomplicated cystitis is very good. With proper
treatment, the infection usually clears up quickly. In many cases, the condition
may reoccur. However, it can be treated in essentially the same way each time it
appears. More complicated infections in men may be difficult to treat if antibiotics
are not able to clear up the problem.

Enuresis (bed wetting)
 Background
 Enuresis is an involuntary passage of urine past the age when a
child should be expected to have attained bladder control
 Etiology
 Unclear
 Possible causes: anatomical malformation of kidneys and bladder;
lack of Anti-diuretic hormone secretion;
mental disorder
 Classification according to time of urine
passage
 Nocturnal- at night
 Diurnal- at morning
 Major types
 Primary- occurs when the child never
establish bladder control
 Secondary- occurs when a person acquires bladder control for the
past 6 months then having relapses and started bed wetting.
 Clinical Findings
 5-7 years old
 Most common in boys
 Laboratory and Diagnostic Findings
 Abnormal ECG patterns
 IVP
 VCUG
 UTz
 Management
 If the cause is stress; stress modification is recommended
 Limit fluids after dinner
 Administration of synthetic Anti-diuretic hormone
 Desmopressin
 Nursing Management
 Urge parents to exercise common sense
 Caution parents of children with sickle-cell anemia not to restrict
fluid because sickling of RBC is increased in case of dehydration
 Advise bladder stretching exercises by drinking lots of water and
trying not to void as long as possible to increase functional size of
the bladder

 Prognosis
 Good if readily prevented
Acute Glomerulonephritis
 Background
Acute Glomerulonephritis is the sudden inflammation of the
glomeruli of the kidney.
 Etiology
 History of recent infection to Group A Beta-Hemolytic
Streptococcus such as: tonsillitis, otitis media, strep throat and
impetigo
 Tissue damage from complement fixation reaction in the
glomeruli
 B- oys
 R- ecent respiratory infection (7-14days)
 A- ges 5-10
 S- pring and winter seasons
 S- trep infection
 Signs and Symptoms
 H- ematuria
 E- dema Periorbital area
 L- ow grade fever
 P-roteinuria
 V-omiting
 A- norexia
 H- eadache
 A-bdominal pain
 CXR & UTz- hepatocardiomegaly, pulmonary edema
 ECG- Galloping HR, T wave inversion, prolong P-R interval,
orthopnea
 Urinalysis: RBC casts, WBC, epithelial cells, hyaline and granules
are present; Increase CRea and BUN
 Blood analysis:
↑Antistreptolysin O
Albumin
Serum Complement
Hct & Hgb
RBC Sedimentation

 Bed rest
 Diet: ↑ Protein;
↓Sodium or DAT with
normal sodium content
 Weighing
 I & O monitoring
 Health teachings
 Positioning semi
fowlers
 Digitalis
 Oxygen therapy

 Nursing Diagnosis
 Situational low self-esteem related to feelings of responsibility for
onset of serious illness.
 Prognosis
 Good if readily prevented
Nephrotic Syndrome
 Background
 Nephrotic syndrome is an abnormal loss of protein in urine
involving immunologic mechanism that may be caused by
hypersensitivity to an antigen-antibody reaction or an immune
process.
 Etiology
 hypersensitivity to an antigen-antibody reaction
 Pediatric Nephrotic Syndrome
1. Congenital- autosomal recessive disorder (rare)
2. Secondary- as progression of glomerulonephritis, sickle cell anemia
or SLE
3. Idiopathic- primary; acquired; common
 Classification of NS according to Membrane
Destruction
 Minimal Change Nephrotic Syndrome
 Focal Segmental Glomerulosclerosis
 Membranoproliferative glomerulonephritis
 Signs and Symptoms
 P- roteinuria
 I- ncrease blood lipid
 D- ecrease serum albumin
 E- dema Periorbital area and abdomen
(ascites)
 + Proteinuria
 Entirely albumin
 Minimal Hematuria
 ↑ RBC sedimentation
 Dx Pocedure: RENAL BIOPSY
 Management

 Supportive-Symptomatic (no cure)
FOCUS: Reducing proteinuria and edema
 Meds: Corticosteroids
IV Methylprednisone: until diuresis w/o protein loss
Oral Prednisone: SE- halt growth and suppress
adrenal gland secretion
 Keeping child free from infection
 Instruct parents to test the first urine of the day
for protein. Approx. 1x a wk.
 Instruct to alternate drug therapy
If diuretics are necessary WOF: tendency for Hypo-K
 Diet: may need to ↑ K & K-supplementation
 Albumin Infusion
 If prednisone resistant (w/ FGS & MPGN):
 cyclophosphamide (anti-neoplastic)
 Mycophenolate
 Cyclosporine
**Rationale: NS is an autoimmune disorder
**Nsg. Consideration: Should take with adequate fluid intake to avoid
bladder irritation and bleeding.
 Nursing Diagnosis
 Imbalance nutrition less than body requirements related to poor
appetite, restricted diet and protein loss
 Prognosis
 MCNS- responds to steroids
 FGS & MPGN- 2 relapses at irregular intervals for several years

Hemolytic-Uremic Syndrome
 Background
 Hemolytic-Uremic Syndrome is the inflammation of glomerular
arterioles because of occlusion with particles of fibrin and platelets.
 Etiology
 Recent E. coli GI infection
 Summer
 6 mos. – 4 years
 Transient diarrhea→ severe fluid
loss and bowel wall
necrosis
 Fever → stupor and hallucination
 Oliguria → proteinuria, hematuria and protein casts
 Edema
 Pale
 Petichiae → thrombocytopenia
 ↑ BUN and Creatinine
 Management
 Symptomatic Approach
 FOCUS: to maintain heart and kidney function
 Oliguria- Peritoneal dialysis
 Anemia- Blood transfusion
 Educate parents about the need for P.D.
 Ensure parents understand the importance of follow up care.
 Prognosis
 Good

Bladder Exstrophy- Epispadia Complex
Background:
Bladder exstrophy-epispadia complex is a congenital abnormality in which part of
the urinary bladder is present outside the body. It is rare, occurring once every
30,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum
of anomalies of the lower abdominal wall, bladder, anterior bony pelvis, and
external genitalia. It occurs due to failure of the abdominal wall to close during
fetal development and results in protrusion of the posterior bladder wall through
the lower abdominal wall.
Etiology:
The cause of bladder exstrophy is maldevelopment of the lower abdominal wall,
leading to a rupture which causes the bladder to communicate with the amniotic
fluid.
Signs and symptoms / Clinical manifestations:
The typical manifestations of exstrophy-epispadias complex are:
 bladder everted through a midline lower abdominal wall defect
 widening of the pubic symphysis
 epispadias in males (dorsal cleft in the penis, exposing the urethral mucosa)
 the anus and vagina appear anteriorly displaced
 The testicles may be undescended.
 Bifid clitoris in females, with a short "urethral strip" indistinguishable from
bladder mucosa.
The spectrum of disease extends from spade penis and epispadias on one hand,
to exstrophy with cloaca (also known as cloacal exstrophy).
Nursing Management:
 In neonates with exstrophy and epispadias, initiate general supportive
care appropriate for the overall condition and associated anomalies.
 Institute parenteral nutrition early for patients with cloacal exstrophy.
 Place clean plastic wrap over the bladder plate. Avoid moistened or
impregnated gauze, which is irritating to the delicate bladder mucosa. Mist
tents may be used to protect exposed tissue.
 Start antibiotic therapy with doctor’s order after delivery and continue
through the early postoperative period.
 Daily prophylactic antibiotic therapy may be continued in the weeks after
bladder closure. Surgeon's philosophy on this matter varies.
 Infections may be related to poor emptying and are to be prevented in light
of the high incidence of vesicoureteral reflux. Institute latex precautions
due to high incidence of latex sensitization in patients with exstrophy-
epispadias complex.

NANDA problems:
Impaired urinary elimination r/t anatomical malformation of the bladder and
ureters
Risk for infection r/t broken skin
Prognosis:
Even with successful surgery, patients may have long-term problems with
 incontinence
 urinary reflux (see Vesicoureteral_reflux)
 repeated urinary tract infections
 bladder adenocarcinoma
 colonic adenocarcinoma
 sexual dysfunction
 pain
 uterine prolapse

Polycystic Kidney Disease
Background:
It occurs in humans and some other animals. PKD is characterized by the
presence of multiple cysts (hence, "polycystic") in both kidneys. The cysts are
numerous and are fluid-filled resulting in massive enlargement of the kidneys.
The disease can also damage the liver,pancreas, and in some rare cases,
the heart and brain. The two major forms of polycystic kidney disease are
distinguished by their patterns of inheritance.
Polycystic Kidney Disease is the most common genetic, life threatening disease
affecting an estimated 12.5 million people worldwide
Etiology:
It is an autosomal recessive trait, and both parents must have carried the gene.
>ESRD
>Uremia (when renal nephrons are destroyed and renal function deteriorates)
>UTI (bec of distorted renal archithecture)
>hyponatremia (PKD tends to waste Na+)
Urinalysis (hema/proteinuria)
CBC (low Hct and Hgb)
Cerebral angiography ((+) aneurysm)
Nursing Management:
1) Aggressive control of HPN
2) Inc sodium intake (but if + HPN, dietary sodium is restricted)
3) Dialysis or renal transplant (if ESRD develops)
4) Genetic counseling (bec of hereditary nature of disease)
NANDA problems:
Pain (acute) related to compression of tissues, trauma to structures from calculi,
inflammation, and infection
Prognosis:
Many infants and children with recessive PKD die from hapatic fibrosis, which
obstructs blood flow and causes bile buildup in the liver. Its symptoms are
enlargement of the liver and the spread of a fibrous connective tissue over the
liver. Those who survive into their 20s may develop splenic, pancreatic, and
vascular problems. Children with recessive PKD often have smaller than average
stature.

Hydronephrosis
Background:
Hydronephrosis is distension and dilation of the renal pelvis calyces, usually
caused by obstruction of the free flow of urine from the kidney, leading to
progressive atrophy of the kidney. In case of hydroureteronephrosis, there is
distention of both the ureter and the renal pelvis and calices
Etiology:
Hydronephrosis is the result of several abnormal pathophysiological occurrences.
Structural abnormalities of the junctions between the kidney, ureter, and bladder
that lead to hydronephrosis can occur during fetal development. Some of these
congenital defects have been identified as inherited conditions, however the
benefits of linking genetic testing to early diagnosis have not been
determined. Other structural abnormalities could be caused by injury, surgery, or
radiation therapy.
Compression of one or both ureters can also be caused by other developmental
defects not completely occurring during the fetal stage such as an abnormally
placed vein, artery, or tumor. Bilateral compression of the ureters can occur
during pregnancy due to enlargement of the uterus. Changes in hormone levels
during this time may also affect the muscle contractions of the bladder, further
complicating this condition.
Sources of obstruction that can arise from other various causes include kidney
stones and blood clots.
The obstruction may be either partial or complete and can occur anywhere from
the urethral meatus to the calyces of the renal pelvis.
Hydronephrosis can also result from the reverse flow of urine from the bladder
back into the kidneys. This reflux can be caused by some of the factors listed
above as well as compression of the bladder outlet into the urethra by prostatic
enlargement or impaction of feces in the colon, as well as abnormal contractions
of bladder muscles resulting from neurological dysfunction or other muscular
disorders.
 onset of intense flank or back pain radiating to the groin,
 nausea, vomiting,
 Sweating.
 Colicky pain
 Blood seen in the urine.
 Chronic hydronephrosis
 weakness,
 malaise

If electrolyte abnormalities occur because the kidneys are unable to regulate
sodium, potassium, and calcium, there may be heart rhythm
disturbances and muscle spasms.
The following laboratory tests may be ordered depending upon what potential
diagnosis is being considered.
 Urinalysis to look for blood, infection or abnormal cells
 Complete blood count (CBC) may reveal anemia or potential infection
 Electrolyte analysis may be helpful in chronic hydronephrosis since the kidneys
are responsible for maintaining and balancing their concentrations in the blood
stream.
 BUN (blood urea nitrogen), creatinine and glomerular filtration rate (GFR) are
blood tests that help assess kidney function.
…Imaging Studies
CT scan of the abdomen can be performed to evaluate the kidney anatomy and
make the diagnosis of hydronephrosis. It also may allow the health care
practitioner to look for the underlying cause including kidney stones or structures
that are compressing the urinary collecting system. Depending upon the situation
and the health care practitioner's concerns, the CT may be done with or without
contrast dye injected into a vein, and with or without oral contrast (that the patient
drinks) to outline the intestine. Most commonly, for kidney stones, neither oral nor
intravenous contrast is needed.
Ultrasound is another imaging study that can be done to look for hydronephrosis.
The quality of the test depends upon the skill of the ultrasonographer to evaluate
the structures in the abdomen and retroperitoneum. Ultrasound is also useful
inwomen who are pregnant where radiation concerns exist.
Intravenous pyelography (IVP) has mostly been replaced by CT scanning but
does have a role in diagnosing some patients and its use is now limited.
KUB X-rays (an X-ray that shows the kidney, ureter, and bladder) are used by
some urologists to classify a kidney stone as radiodense or radiolucent and may
use KUB X-rays to determine if the stone is able to migrate down the ureter into
the bladder.
Nursing Management:
1) Asses:
Pain
Urine input& output
Palpate kidney
Urine for labs (spec gravity, albumin, glucose, and edema)
2) Intervention:
Administer fluids (hourly fluid replacement)
Watch out for pain and reduced U.O.

Avoid urinary infections(keep urine bag above not touching the floor)
NANDA problems:
Impaired urinary elimination r/t obstruction (mechanical or anatomical)
Risk for infection r/t urinary stasis
Prognosis:
Left untreated, bilateral obstruction (occurring to both kidneys rather than one)
has a poor prognosis

Wilm’s Tumor
Background:
Wilm’s Tumor or nephroblastoma is cancer of the kidneys that typically
occurs in children, rarely in adults. Its common name is an eponym, referring to
Dr. Max Wilms, the German surgeon (1867–1918) who first described this kind of
tumor.
Approximately 500 cases are diagnosed in the U.S. annually. The majority
(75%) occurs in otherwise normal children; a minority (25%) is associated with
other developmental abnormalities. It is highly responsive to treatment, with
about 90% of patients surviving at least five years.
Etiology:
Wilms tumor may arise in 3 clinical settings, the study of which resulted in
the discovery of the genetic abnormalities that lead to the disease. Wilms tumors
can arise sporadically, can develop in association with genetic syndromes, or can
be familial. Although some of the molecular biology of Wilms tumor is coming to
light, the exact cellular mechanisms involved in the etiology of the tumor are still
being investigated.
...More common findings:
 Palpable abdominal mass
 Gross Hematuria (onset: 9 mos), Flank pain, Fever , Weight loss,
Cachexia fatigue, HPN, amyloidosis, thromblophlebitis,
anemia,erythrocytosis, hypercalcemia, abnormal serum liver profile,
elevated ESR,
…Less frequent findings:
 Peripheral neuropathy, inferior vena cava obstruction, priapism, variocele,
hydronephrosis (if tumor blocks ureteropelvic junction)
Plasma erythropoietin, renin, chorionic gonadotropin ang prostaglandin are
elevated
IV Pyelogram
UTZ
CT Scan
Nephrotomography
Nursing Management:
PRE- OP: Inc fluid intake if indicated, emotional support
POST-OP:
V/S, WOF signs of hemorrhage, pneumothorax

NANDA problems:
Anxiety r/t threat of death
Risk for injury
Interrupted family process r/t expensive treatments
Knowledge deficit of the disorder and therapy
Prognosis:
Tumor-specific loss-of-heterozygosity (LOH) for chromosomes 1p and 16q
identifies a subset of Wilms' tumor patients who have a significantly increased
risk of relapse and death. LOH for these chromosomal regions can now be used
as an independent prognostic factor together with disease stage to target
intensity of treatment to risk of treatment failure. Genome-wide copy number and
LOH status can be assessed with virtual karyotyping of tumor cells (fresh or
paraffin-embedded). The overall prognosis with surgical removal is positive. Early
removal tends to promote positive outcomes.

Hypertrophic Pyloric Stenosis
A condition that causes severe vomiting in the few months of life. There is
narrowing (stenosis) of the opening from the stomach to the intestines, due to
enlargement (hypertrophy) of the muscle surrounding this opening (pylorus),
which spasms when the stomach empties. It is uncertain whether there is a real
congenital narrowing or whether there is a functional hypertrophy of the muscle
which develops few weeks of life.
Hypertrophic pyloric stenosis may also cause almost complete gastric
outlet obstruction. It affects 1 of 250 infants and is common among males by a
4:1 ratio, particularly firstborn males. It occurs most often between 3 to 5 weeks
of age and rarely after 12 weeks.
Etiology
There is no known etiology of pyloric stenosis, but a genetic component is
likely because siblings and offspring of affected people are at increased risk.
Proposed mechanisms include lack of neuronal nitric oxide synthase and
abnormal innervations of the muscular layer. Infants exposed to certain
macrolide antibiotics in the first few weeks of life are at significantly increased
risk.
Clinical Findings
- Olive shaped mass
palpated on the
epigastrium.
- Palpable or even visible
peristaltic waves
- Thickened pylorus in
ultrasound
- Narrowed pyloric outlet in
upper GI series
- Hypokalemic,
hypochloremic metabolic
alkalosis in blood
chemistry
- Hypovolemia
- Hyperaldosteronism
Signs and Symptoms
- Progressive worsening
non-bile stained projectile
vomiting
- Poor feeding
- Weight loss
- Crying without tears and
less wet or dirty diapers
(dehydration)
- Constant hunger
- Belching
- Colic
Laboratory and Diagnostic procedures
- Ultrasound ( most common diagnostic exam)
- Upper gastro-intestinal series ( most common confirmatory diagnostic
exam)
- Blood chemistry and arterial blood gases
Nursing Management
- Immediate fluid replacement (dehydration and electrolyte imbalances)

- Oral atropine
- If the client undergoes surgery, secure consent and properly educated
the client’s parents and guardians about the procedure.
- If the client has an ostomy, clean the stoma properly and regularly, and
educated the client’s parents and guardians in how to clean the stoma.
Nursing Diagnosis
- Fluid volume deficit
- Imbalanced nutrition less than body requirement
- Risk for aspiration
Prognosis
If intervention and surgery are immediately done, good prognosis is
accomplish.

CLEFT LIP (CL) AND/ OR CLEFT PALATE (CP)
Cleft lip and cleft palate are facial malformations that occur during
embryonic development and can constitute a severe disability to the affected
individual. Cleft lip, also known as cheiloschisis, and cleft palate, also known
as palatoschisis, are types of abnormal developments of the face during
pregnancy - they are types of clefting congenital deformities. They can occur
together as cleft lip and palate.
Before birth, there are natural
structures that form in the body and
then join together (fuse). A cleft is a
non-fusion of these structures - a
fissure or a gap. In this case, the gap
(cleft) occurs in the upper lip, the
palate (roof of the mouth), or both.
When just one side of the lip is
affected it is called a unilateral cleft;
a bilateral cleft affects both sides.
According to the National Institutes
of Health (NIH), USA, about 1 in
every 700 newborns has a cleft lip
and/or cleft palate. The National
Health Service (NHS), UK says the
incidence is 1 in 600.
ETIOLOGY
Researchers believe that most cases of cleft lip and cleft palate are caused by an
interaction of genetic and environmental factors. In many babies, a definite cause
isn't discovered.
 Genetic factors. Either the mother or the father can pass on genes that cause
clefting, either as an isolated defect or as part of a syndrome that includes
clefting as one of its signs. In some cases, babies inherit a gene that makes them
more likely to develop a cleft, and then an environmental trigger actually causes
the cleft to occur.
 Environmental factors. Fetal exposure to cigarette smoke, alcohol, certain
medications, illicit drugs and certain viruses have been linked to the development
of a cleft.
Other risk factors:
Several factors may increase the likelihood of a baby developing a cleft lip and
cleft palate.

 Family history. Parents with a family history of cleft lip or cleft palate face a
higher risk of having a baby with a cleft.
 Race. Cleft lip and palate are most common in American Indian and Asian
children. Black children are least likely to have a cleft.
 Sex. Males are twice as likely to have a cleft lip with or without cleft palate. Cleft
palate without cleft lip is more common in females.
 Environmental factors. Exposure in early pregnancy to cigarette smoke, alcohol
or illicit drugs may put a baby at higher risk of developing a cleft.
 Maternal obesity. Obesity in the mother is associated with a slightly increased
risk of cleft lip and palate.
PATHOPHYSIOLOGY
During embryonic development the lateral and medial tissues forming the
upper lip palates fuse between weeks 7 and 8 of gestation; the palatal tissues
forming the hard and soft palates fuse between weeks 7 and 12 gestation. Cleft
lip and cleft palate result when these tissues fail to fuse.
MANIFESTATIONS
Clinical manifestations
a. Cleft lip and cleft palate are readily apparent at birth. Careful physical
assessment should be performed to rule out other midline birth defects. Palpate
the palate with the fingers to check for defects.
b. Cleft lip and cleft palate appear as incomplete or complete defects, and may
be unilateral or bilateral.
Laboratory and diagnostic study findings.
Obstetric ultrasound will reveal cleft lip while the infant is in utero at 18 to 20
weeks of gestation can identify a cleft in a fetus.
COMPLICATIONS
Children with cleft lip with or without cleft palate face a variety of challenges,
depending on the type and severity of the cleft.
 Feeding difficulties. One of the most immediate concerns after birth is feeding.
While most babies with cleft lip can breast-feed, a cleft palate can make sucking
difficult or cause gagging or nasal regurgitation. Your health care team will
discuss feeding strategies with you, such as using a special bottle nipple or a
small artificial palate (obturator) that fits into the roof of the mouth.
 Ear infections and hearing loss. Babies with cleft palate are especially
susceptible to middle ear infections. Over time, repeated ear infections can
damage hearing, but hearing loss may resolve with treatment. It's important for
children with cleft palate to be evaluated regularly by an audiologist or an ear,
nose and throat doctor. Most children with clefts have tubes inserted in their ears
to drain fluids and help prevent infections.

 Dental problems. If the cleft extends through the upper gum, tooth development
will likely be affected. A pediatric dentist should monitor tooth development and
oral health from an early age.
 Speech difficulties. Because both the lip and palate are used in forming
sounds, the development of normal speech can be affected. A speech
pathologist can evaluate your child and provide speech therapy.
 Psychological challenges. Children with clefts may face social, emotional and
behavioral problems due to differences in appearance and the stress of intensive
medical care. A psychologist and a social worker can help you and your child
deal with the stresses your family encounters.
NURSING MANAGEMENT
Assess for problems with feeding, breathing parental bonding, and speech.
Provide child and family teaching.
Ensure adequate nutrition and prevent aspiration.
a. Provide special nipples or feeding devices (eg, soft pliable bottle with soft
nipple
with enlarged opening) for a child unable to suck adequately on standard
nipples.
b. Hold the child in a semi-upright position; direct the formula away from the
cleft and toward the side and back of the mouth to prevent aspiration.
c. Feed the infant slowly and burp frequently to prevent excessive swallowing
of air and regurgitation.
d. Stimulate sucking by gently rubbing the nipple against the lower lip.
Feeding bottle for infant with Cleft lip and cleft palate.
Support the infant’s and parents’
emotional and social adjustment.
a. Help facilitate the family’s
acceptance of the infant by
encouraging the parents to
express their feelings and
concerns and by conveying

an attitude of acceptance
toward the infant.
b. Emphasize the infant’s positive
aspects and express optimism
regarding surgical correction
.
Provide preoperative care.
a. Depending in the defect and the child’s general condition, surgical
correction of the cleft lip (cheiloplasty ) usually occurs at 1 to 3 months
of age; repair of the cleft palate (palatoplasty) is usually performed
between 6 and 18 months of age. Repair of the cleft palate may require
several stages of surgery as the child grows.
b. Early correction of cleft lip enables more normal sucking patterns and
facilitates bonding. Early correction of cleft palate enables development
of more normal speech patterns.
c. Delayed closure or large defects may require the use of orthodontic
appliances.
d. The responsibilities of the nurse are to:
1. Reinforce the physician’s explanation of surgical procedures.
2. Provide mouth care to prevent infection.
Provide postoperative care.
a. Assess airway patency and vital signs; observe for edema and respiratory
distress.
b. Use a mist tent, if prescribed, to minimize edema, liquefy secretions, and
minimize distress.
c. Position the child with cleft lip on her back, in an infant seat, or propped on
a side to avoid injury to the operative site; position the child with a cleft
palate on the abdomen to facilities drainage.
d. Clean the suture line and apply an antibacterial ointment as prescribed to
prevent infection and scarring. Monitor the site for signs of infection.
e. Use elbow restraints to maintain suture line integrity. Remove them every 2
hours for skin care and range-of-motion exercises.
f. Feed the infant with a rubber-tipped medicine dropper, bulb syringe, Breck
feeder, or soft bottle-nipples, as prescribed, to help preserve suture
integrity. For older children, diet progresses from clear fluids; they should
not use straws or sharp objects.
g. Attempt to keep the child from putting tongue up to palate sutures.
h. Manage pain by administering analgesic as prescribed.
NURSING DIAGNOSIS
 Altered nutrition : less than body requirements related to physical defect
 Risk for altered parenting related to infant with a highly visible physical
defect

 Risk for trauma of the surgical site related to surgical procedure,
dysfunctional swallowing
 Altered nutrition: less than body requirements related to difficulty eating
following surgical procedure
 Pain related to surgical procedure
 Altered family processes related to child with a physical defect,
hospitalization
PROGNOSIS
Although treatment may continue for several years and require several
surgeries, most children with a cleft lip and palate can achieve normal
appearance, speech, and eating. However, some people may have a continued
speech problem that needs further therapy.
CLEFT LIP/ CLEFT PALATE MIND MAP

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA
. A rare congenital malformation that is believed to result from failed separation of
the esophagus and trachea by a septum that forms in the 4th week of gestation.
The esophagus ends in a blind pouch and is accompanied by
Tracheoesophageal Fistula.
ETIOLOGY AND INCIDENCE
The cause of esophageal atresia
and TEF is unknown.
• 1:2000 to 1:1500 live births.
• Happens in both sexes.
• EA/TEF is often present in
VATER or VATERL
syndromes
• V-ertebral
• A-norectal
• C- ardiovascular,
• T-racheo
• E-sophageal
• R-enal
• L-imb abnormalities
• Low birth weight babies.
• And high incidence in
premature infants
TYPES:
 Type A (7.7%): Esophageal atresia in which both segments of the
esophagus end in blind pouches. Neither segment is attached to the
trachea.
 Type B (0.8%): Esophageal atresia with tracheoesophageal fistula in
which the upper segment of the esophagus forms a fistula to the trachea.
The lower segment of the esophagus ends in a blind pouch. This condition
is very rare.
 Type C (86.5%): Esophageal atresia with tracheoesophageal fistula, in
which the upper segment of the esophagus ends in a blind pouch (EA)
and the lower segment of the esophagus is attached to the trachea (TEF).
 Type D (0.7%): Esophageal atresia with tracheoesophageal fistula, in
which both segments of the esophagus are attached to the trachea. This
is the rarest form of EA/TEF.
 Type H (4.2%): Tracheoesophageal fistula in which there is no
esophageal atresia because the esophagus is continuous to the stomach.
Fistula is present between the esophagus and the trachea.

SIGN AND SYMPTOMS
• Excessive salivation or drooling
• Three C’s OF TEF
• C-hoking
• C-oughing
• C-yanosis
• Apnea
• Increased respiratory distress after feeding
• Abdominal distention
DIAGNOSTIC EXAM
• Esophageal Atresia- maternal polyhydramnios.
• Determined by radiographic studies
• A size 10 or 12 French catheter passed through the nose meets an
obstruction (esophageal atresia) approximately 4" to 5" (10 to 12.5 cm)
distal from the nostrils. Aspirate of gastric contents is less acidic than
normal.
• Chest X-ray demonstrates the position of the catheter and can also show
a dilated, air-filled upper esophageal pouch, pneumonia in the right upper
lobe, or bilateral pneumonitis. Both pneumonia and pneumonitis suggest
aspiration.
• Abdominal X-ray shows gas in the bowel in a distal fistula (type C) but
none in a proximal fistula (type B) or in atresia without fistula (type A).
• Cinefluorography allows visualization on a fluoroscopic screen. After a
size 10 or 12 French catheter is passed through the patient’s nostril into
the esophagus, a small amount of contrast medium is instilled to define
the tip of the upper pouch and to differentiate between overflow aspiration
from a blind end (atresia) and aspiration due to passage of liquid through
a tracheoesophageal fistula.
COMPLICATIONS
 The infant may breathe saliva and other secretions into the lungs, causing
aspiration pneumonia, choking, and possibly death.
 Other complications may include:
 Feeding problems
 Reflux (the repeated bringing up of food from the stomach) after
surgery
 Narrowing (stricture) of the esophagus due to scarring from surgery
 Tracehomalacia – weakness in the tracheal wall that occurs when a
dilated proximal pouch compresses the trachea in early fetal life.
 Prematurity may complicate the condition.

THERAPEUTIC/NURSING MANAGEMENT
• Keep infant warm and oxygenated
• Keep infant supine with the HOB elevated to keep gastric secretions from
entering the lungs
• NGT aspirate every 5 to 10 minutes to keep the keep the proximal pouch
clear
• Intravenous IV fluids are essential
• Surgical repair: Ligation of the fistula and end-to-side anastomosis of the
atresia.
NURSING DIAGNOSIS
 Impaired gas exchange and ineffective airway clearance related to
Respiratory distress/ EA& TEF
 Risk for aspiration related to infants immature gag and cough reflex
 Risk for imbalanced nutrition: less than body requirements related to
inability to take in oral feedings
 Risk for infection related to aspiration or seepage of stomach secretions
on the lungs
 Risk for impaired skin integrity related to gastrostomy tube insertion site.
PROGNOSIS
Surgery to correct esophageal atresia is usually successful, with
survival rates close to 100 percent in otherwise healthy infants after the
condition is corrected. Postoperative complications may include difficulty
swallowing, since the esophagus may not contract efficiently, and

gastrointestinal reflux, in which the acidic contents of stomach back up
into the lower part of the esophagus, possibly causing ulcers.

BACKGROUND OF THE DISEASE
Intussusception- the invagination of one portion of the intestine into another,
usually occurs in the second half of the first year of life.
ETIOLOGY
<1 year old-occurs for Idiopathic reasons
>1 year old-a “lead point” on the intestine likely cues the invagination
Meckel’s diverticulum-a polyp, hypertrophy of Peyer’s patches (lymphatic tissue
of the bowel that increases in size with viral diseases) or bowel tumors. The point
of the invagination is generally at the juncture of the distal ileum and proximal
colon.
SIGNS AND SYMPTOMS
Assessment:
 Suddenly draw up their legs and cry and they vomit
 After the peristaltic wave, they are symptom free and play happily
 (Approx. 15 min.) Same phenomenon of intense abdominal pain strikes
again
 Vomitus will begin that contains bile bec. the obstruction is invariably
below the ampulla of Vater (the point in the intestine where bile empties
into the duodenum
 (Approx. 12 hrs.)blood appears into the stool and possibly in vomitus
(currant jelly appearance)
 The abdomen becomes distended as the bowel above the
intussusceptions distends.
DIAGNOSTIC TEST
The presence of intussusceptions is confirmed by ULTRASOUND or CT scan
MANAGEMENT
 Surgical Emergency
 Instillation of a water soluble sol’n, barium enema or air (pneumatic
insufflation) into the bowel or surgery to reduce invagination.
 After this type of reduction, children must observe for 24 hrs. because
some children will have recurrence of the intussusceptions.
NURSING DIAGNOSIS
 Pain related to abnormal abdominal peristalsis
 Risk for deficient fluid volume related to bowel obstruction
 Risk for impaired parenting related to infant’s illness

Hirschsprung’s Disease-is absence of ganglionic innervations to the muscle of
a section of the bowel. In most instances, the lower portion of the sigmoid colon
just above the anus. The absence of nerve cells means there are no peristaltic
waves in this section to move fecal material through the segment of the intestine.
ETIOLOGY
Assessment:
 If infants fail to pass
meconium by 24
hours of age
 Increasing
abdominal
distention
 History of
constipation or
intermittent
constipation and
diarrhea
 What is the duration of the constipation? (It may have been a
problem from birth)
 What do parents mean
constipation? (Children do not have
a bowel movement more than a
week)
 What is the consistency of the
stool? (Ribbonlike or watery)
 Is the child ill in any other way?
(Children with aganglionic disease
of the intestine tend to be thin and
undernourished, sometimes
deceptively so because their
abdomen is large and distended)
Normal Hirschsprung Disease
If a gloved finger is inserted into the
rectum of a child with true constipation, the
examining finger will touch hard, caked
stool.
With aganglionic disease, the rectum is
empty because fecal material cannot pass
into the rectum through the obstructed
portion.

DIAGNOSTIC TEST
 A barium enema is generally ordered to substantiate the diagnosis. The
barium will outline on x-ray film the narrow, nerveless portion and the
proximal distended portion of the bowel
 Biopsy of the affected segment to show the lack of innervations or by
anorectal manometry (a technique to test the strengthor innervations of
the internal rectal sphincter by inserting a balloon catheter into the rectum
and measuring the pressure exerted against it.
MANAGEMENT
SOAVE PULL THROUGH OPERATION
Repair of aganglionic megacolon involves dissection and removal of affected
section, with anastomosis of the intestine (termed a pull-through operation)

Two stage surgery:
 Temporary colostomy
 Bowel repair at 12 to 18 months of age
*After the final surgery, the children should have functioning, normal bowel. In the
few instances in which the anus is deprived of nerve endings, a permanent
colostomy will established.
NURSING DIAGNOSIS
 Constipation related to reduced bowel function
 Imbalanced nutrition, less than body requirements, related to reduced
bowel function

Anorectal Malformation-are birth
defects (problems that happen as a
fetus is developing during
pregnancy). With this defect, the
anus and rectum (the lower end of
the digestive tract) do not develop
properly. "Ano" refers to the anus
(the opening at the end of the large
intestine through which stool passes
when a baby has a bowel
movement) "Rectal" refers to the
rectum (the area of the large
intestine just above the anus)
With an anorectal malformation, several abnormalities can occur, including the
following:
 a membrane may be present over
the anal opening
 the rectum may not connect to the
anus (imperforate anus)
 the rectum may connect to a part
of the urinary tract or the
reproductive system through an
abnormal passage called a fistula
Diagnostic test: Abdominal ultrasound/sonography and xray
MANAGEMENT
Treatment may depend on the ff:
 the extent of the problem
 the overall health of the baby and the medical history
 parental opinion and preference
 the opinion of the physicians involved in the baby's care
 expectations for the course of the disease
The majority of babies with anorectal malformation will need to have surgery to
correct the problem. The type and number of operations necessary depends on
the type and extent of abnormality the baby has, including the following:
 Narrow anal passage - Babies who have the type of malformation that
causes the anal passage to be narrow may not need an operation. A
procedure known as anal dilatation may be done periodically to help
stretch the anal muscles so stool can pass through it easily. However, if
the anal opening is positioned wrongly, an operation may be neeeded to
correctly relocate the anal opening.
 Anal membrane - Babies with this type of malformation will have the
membrane removed during surgery. Anal dilatations may need to be done

afterward to help prevent any narrowing of the anal passage that is
present.
 Lack of rectal/anal connection, with or without a fistula -These babies may
need a series of operations in order to have the malformation repaired.
IMPERFORATE ANUS
Imperforate Anus is stricture of anus. It is
congenital (present from birth) defect in
which the opening to the anus is missing or
blocked.
Etiology/ cause
The problem is caused by abnormal
development of the fetus. In week 7 of
intrauterine life, the upper bowel elongates
to pouch and combine with a pouch
invaginating form the perineum
Clinical findings
 It is a relatively common condition that occurs in about 1 out of 5,000
infants.
 Most common in boys
Sign and symptoms
 Anal opening very near the
vaginal opening in girls
 Missing or misplaced opening
to the anus
 No passage of first stool
within 24 - 48 hours after birth
 Stool passes out of the
vagina, base of penis,
scrotum, or urethra
 Swollen belly area
Diagnostic/ laboratory
 Prenatal sonogram, Radiograph
Assessment
 Inspection of the newborn’s anal region
 May be revealed because a membrane filled with black meconium

 No wink reflex in the anal area (touching the skin near the rectum should
make it contact)
 Inability to insert rubber catheter into the
rectum
Nursing management
 Anastomosis, Colostomy
NANDA problems
 Imbalanced nutrition, less than body
requirements, related to bowel obstruction
and inability for oral intake
 Impaired tissue integrity at rectum related to
surgical incision
 Risk for impaired parenting related to difficulty in bonding with infant ill
from birth
GASTRO ESOPHAGEAL REFLUX
DISEASE
Gastro esophageal reflux disease is a
condition in which the liquid content of the
stomach regurgitates (backs up or refluxes)
into the esophagus. The liquid can inflame
and damage the lining of the esophagus
although visible signs of inflammation occur
in a minority of patients
Etiology/ cause
 Neuromuscular disturbance in which the cardiac sphincter and the lower
portion of the esophagus spasm and allow easy regurgitation of gastric
contents into the esophagus
 Incompetent cardiac sphincter
 Maybe related to hiatal hernia
Clinical findings
 Starts within 1 week after birth
 Regurgitation starts after feeding
Sign and symptoms
 Effortless vomiting not
projectile
 heartburn, regurgitation, and
nausea.

 Ulcers, strictures, esophageal
cancer
 Inflammation of larynx and
throat
 Inflammation and infection of
lungs
 Fluid in the sinuses and
middle ears
Diagnostic and laboratory
 Endoscopy (fiberoptic
endoscopy) esophagography
 Esophageal ph testing
 X-ray
 Examination of throat and
larynx
 Esophageal manometry- used
to measure the strength of
the esophageal sphincter
Nursing management
 Formula or breast milk with
rice cereal
 Upright position while eating
and an hour after eating
 Laparoscopic or surgical
myotomy (narrowing of the
esophageal sphincter)
 Ranitidine
 Omeprazole
 Avoid acidic, fatty and
alcoholic foods
 Eat small portion of foods
NANDA problems
Risk for imbalanced nutrition, less than body requirements, related to
regurgitation of food with esophageal reflux
CONSTIPATION
Difficulty of passing hardened stools, may occur in children of
any age
Cause/ etiology
 Lack of fluid intake
 High fiber meals
Clinical findings
 Distressing to children (painful, and may have anal fissures)
 May experience abdominal pain from force of intestinal contractions
 Large and firm stool
Sign and symptoms
 Hard with discomfort in defecating

 Anal fissures (may cause blood in
stool)
 Abdominal pain
Diagnostic and laboratory findings
 Blood occult test (for sign of
bleeding)
Nursing management
 Increase fluid intake
 Diet high in fiber
 Privacy in bathroom
 Determine the cause of stress
NANDA problems
Constipation related to pain from anal fissure and hardened stool
DIARRHEA
Frequent bowel evacuation or the passage of abnormally soft of liquid feces
Etiology/cause
 Caused by virus which is the major
cause of infant gastroenteritis
 Common viral pathogens : rotavirus,
adenovirus
 Common bacterial pathogens ;
campylobacter jejuni, salmonella, giardia
lamblia and clostridium difficile
 Acute associated by infection
 Chronic associated with malabsorption and inflammatory cause
Sign and symptoms
 Frequency- unlimited number
 Color- green
 Effort of expulsion- effortless; may be
explosive
 pH- less than 7 (acidic)
 Odor- sweet or foul
 Occult blood- positive; blood may be overt

 Reducing saubstances- positive
Assessment (mild diarrhea)
 Anorectic and irritable
 Episodes of diarrhea 2-10
loose watery
 With fever (38.4-39)
 Warm skin
 Dry mouth
 Rapid pulse
Assessment (severe diarrhea)
 39.5-40 temperature
 Pulse and respiration are
weak and rapid
 Pale and cool skin
 May appear apprehensive,
listless and lethargic
 Depressed fontanelle, sunken
eyes and poor skin turgor
 Elevated hemoglobin and
hematocrit because of
dehydration
 Loss in body weight
Therapeutic management
 Oral rehydration solution
 Rest the GI tract
 Breastfeeding
 Zinc administration (zinc deficiencies)
 Antibiotic therapy
NANDA problems
Deficient fluid volume related to loss of fluid through diarrhea
Risk for impaired skin integrity related to presence of diarrheal stool on skin
APPENDICITIS
Appendicitis is a condition characterized by inflammation of the
appendix. It is classified as a medical emergency and many cases require
removal of the inflamed appendix, either by laparotomy or laparoscopy.
Untreated, mortality is high, mainly because of peritonitis and shock
 First visible during week 8
 Function Unknown
 Most likely caused by luminal obstruction
ETIOLOGY:
 Fecal material

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