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General Objective

By the end of the class the student will be


able to identify different congenital
anomalies that are present at birth
Specific Objectives:
List the possible causes of fetal malformations.
Define some common congenital anomalies in the
newborn infant.
Describe some of the surgery related differences
between infants and adults.
Outline important aspects in the pre and post
operative care of pediatric patients.
Discuss specific pre and post operative care of a child
with the following: cleft lip, cleft palate, esophageal
atresia and pyloric stenosis.
Causes of Fetal Malformation

Genetic Traits
Viruses

Drug
Radiation
Surgery Related Differences between Young
Children and Adults

 The metabolic rate of the infant and


young children is much greater
proportionately than that of adult.
 The body tissues of the child heal
quickly because of his rapid rate of
metabolism and growth.
 The child usually needs proportionately less
analgesic than adult patient to obtain relative
comfort after surgical procedures.
 The child lacks the reserve physical resources
that are available to the adult. His general
condition may change very rapidly , almost
without warning.
 Abnormal fluid loss is more serious in the infant
and young child than in the adult. Fluid intake
and output must be calculated very carefully.
General Aspects of
Pre & Post-Operative Pediatric Care

Some newborns with birth


defects are critically ill.
They may need to be
transported to medical
centers or specialized
pediatric hospitals.
Thus if acutely ill infant is to be transferred,
provision should be made to ensure
that the trip will be safe and that
there will be no appreciable
deterioration of the infant’s condition
◈ Safe means of transportation with a
heated portable incubator and
available oxygen supply is needed so as
to maintain the infant’s body
temperature and O2 therapies in the
blood.
◈ Equipment for suctioning to remove
secretions is needed (as in esophageal
atresia).
◈ A nurse should participate in the
transfer of the baby to observe him
during that time and give appropriate
care in emergency (e.g. prevent
torsion of an omphalocele, proper
position of infant).

◈ All pertinent infant information should


accompany the infant as he goes from
one health agency to another
Pre-operative
care
 Psychological preparation of the child
(according to his age). This aimed to
prevention of fears common to children like
fear of separations, fear of death.

 The nurse should explain to the child and


his families what is going to occur.

 Except in emergency situations, children


should preferably be free of respiratory
complications and signs of malnutrition.
Most children must have nothing by mouth
before surgery but the length of the time
the child remains NPO will depend on age
(for newborns and infants 3-4 hours is
usually enough).
The incision over or the part involved in
surgery must be washed and inspected.
Shaving may be needed.
The mouth should be checked for loose
teeth or for dentures (particularly in
children of 6-8 years of age). Any loose or
missing teeth should be charted in the
child’s record.
Remove barrettes and pins from the child’s
hair.
Clothing should be warm and loose. The
child should be dressed in a hospital gown
and under pants only.
Check the child’s identification band to see
that is legible and secure. If not it needs to
be replaced prior to surgery.
Pre-medication: sedatives and analgesics
are usually given two hours before surgery
except in emergency situation.
 The nurse should chart whether the child
has passed urine and had a bowel movement.
Enemas are never done routinely but may be
ordered in some conditions.
 Prior to taking the child to surgery,
especially in newborns and infants, nostrils
should be carefully cleansed (hard crusts
may be softened with a solution of sodium
bicarbonate, normal saline, or even warm
water). This is necessary to because crusts
may be obstructing the airway.
The child may need to take his favorite
toy with him to surgery. Ideally, he
should be allowed to keep the toy with
him until he is under the anesthetic.

Parents should be allowed to


accompany their children to the
operation site if they so desire.

Parents should be told where to wait


during the surgery, whether the child will
go to recovery room after surgery or
directory to his unit.
After return from the operating room, the
child’s general condition must be closely
observed
a) Vital signs, especially temperature.
b) Airway must be kept patent newborn
babies must be kept in warm cot or
incubator.
 Unit the child is responsive and
alert; he should be kept on his side
(for secretion and vomitus to get
out from mouth).

 Observe conditions and placement


of dressing. Check and mark any
apparent drainage from wound.

 Intravenous fluids should be


checked for correct rate of flow
and for possible infiltration.
The child should be carefully handled and
should be protected from harming himself by
use of appropriate restraints.
Any urinary catheter should be connected to
drainage bag and stabilized properly to bed.
Observe patient’s skin color and temperature
as well as any sings of shock :
(a) low blood pressure
(b) rapid pulse
(c) cold moist pale or cyanotic skin
(d) dilated pupils
(e) restlessness.
 Oral fluids may be started often after the
following criteria are observed (a) color of
aspirate is clear (b) peristaltic movements
are heard (c) flatus or gases are passed.
 Oral fluids should be started while infusion
still on. If well tolerated then infusion is
gradually discontinued.
 Routine postoperative diet is modified
according to child’s age, but in general it
changes from clear of liquid, full liquid, soft
and then regular diet.
 Sedatives are used according to prescribed
orders and child’s needs.
• For children who can walk, early progressive
ambulation is the rule (expect in few cases),
this will help to restore GIT function and
prevent complications of pneumonia, the
thrombosis, and pressure areas.
• If too young to go out of bed, the nurse
should turn the child frequently and give him
good skin care and help him to breath deeply
at intervals.
☆It is defined as a structural
defect present at birth.

☆Some anomalies are


compatible with life and
treatment could be delayed,
while others are incompatible
with life and must be
repaired immediately
Gastrointestin
al Anomalies
Anomalies of the Mouth

It is a congenital fissure in the upper lip. It is


generally located at one side or the other of
the midline in the center of the nares. It may
be unilateral or bilateral
It is incomplete fusion of the palatal shelves.
The opening may involve only the soft palate
or the hard palate or both.
It may be a single midline cleft or two
bilateral clefts.
Cleft palate may occur in connection with
cleft lip
Cause:
Both cleft lip and palate result from failure of
or incomplete union of embryonic structures
of the face.
Clinical Manifestations

Observable defects.
Complications associated with cleft lip
and cleft palate
 Feeding difficulties
 Ear infections and hearing loss
 Speech and language delay
 Dental problems
Feeding baby with cleft palate
 Breast-feeding is allowed. It will take extra time
and patience.
 Hold an infant in an upright position to help
keep the food from coming out of the nose.
 Small, frequent feedings are recommended.
 There are many types of bottles and nipples that
can assist with feeding an infant with cleft
palate.
–NUK nipple
This nipple can be placed on regular bottles or
on bottles with disposable bags. The hole can
be made larger by making a criss-cross cut in
the middle.
Mead Johnson Nurser®
This is a soft, plastic bottle that is easy to
squeeze and has a large crosscut nipple.
Haberman Feeder®
This is a specially designed bottle system with a
valve to help control the air the baby drinks and
to prevent milk from going back into the bottle.
Neonatal Respiratory
Distress

Oesophageal Atresia with


Congenital
Tracheoesophageal
Diaphragmatic Hernia
Fistula
Esophageal Atresia and
Tracheoesophageal Fistula: (T.E.F.)

 It is congenital
malformation represent
a failure of the
esophagus to develop
a continuous passage.
 These defects may
occur separately or in
combination.
Definition
 Oesophageal atresia is defined: as an
interruption in the continuity of the
esophagus with or without fistula to the
trachea.
 Tracheoesophageal Fistula is defined
as abnormal fistulous communication
between the trachea and the esophagus
with Atresia of the esophagus singly or in
combination.
Etiology:
Unknown.
Types:

 Oesophageal
Atresia with a distal
tracheo-
oesophageal fistula

 Oesophageal
Atresia with a
proximal fistula
Oesophageal Atresia: (con‘t)
 Oesophageal atresia
combined with fistulae
from both
oesophageal
segments

 Isolated Oesophageal
Atresia without a
fistula
 V = Vertebral anomalies

 A = Anal anomalies

 TE = Tracheoesophageal Fistula

 R = Renal deformaties
Three Cs of TEF on feeding:
• Coughing.
• Choking.
• Cyanosis.
• and, regurgitation
 Prematurity and polyhydramnios are
common association.
 Excessive salivation and drooling
 Apnea.
 Increased respiratory distress following
feeding.
 Abdominal distention.
 Regurgitation of saliva and milk from
the obstructed upper pouch into the
trachea.
 Reflux of acidic content through the
fistula into the lung leading to
pneumoniatis.
 Gastric distention by air from trachea
leading to diaphragmatic elevation.
 Insertion of catheter gently into the
esophagus meets with resistance if the
lumen is blocked but passes unobstructed if
the lumen is patent.
 Radiographic visualization of opaque
catheter inserted into the hypopharynx
helps to determine patency and / or
alternate channels.
 Endoscopy for isolated fistula.
• Anastmosis  Leakage ,Stricture
and recurrent.
• Pleural  Pnumothorax and
empyema.
• Dysphagia  From disordered
esophageal peristalsis.
Congenital Diaphragmatic Hernia
 The cause of CDH is largely
unknown.
 CDH can occur as part of a
multiple malformation
syndrome in up to 40% of
infants (cardiovascular,
genitourinary, and
gastrointestinal malformations)
Congenital diaphragmatic hernia
(CDH) is a malformation
characterized by a defect in the
Posterolateral diaphragm,
through which the abdominal
viscera migrate into the chest
during fetal life.
 Most common type due to early reduction of
physiological hernia (normal at the 10th week)
before closure of the foramen of Bochdalek by
the pleuroperitoneal membrane at the 8th
week.

 It is more common on the Lt. Side because the


left side usually closes later than the Rt. Side
does. The sac is absent in 90% of cases.
What is it?
1) Failure of diaphragmatic fusion
2) Herniation of abdominal contents into
thoracic cavity
3) Pulmonary hypoplasia
What is it?
Diagnosis
Diagnosis of CDH is made postnatally by plain
radiography of the chest and abdomen by
demonstration of:
 Air-filled loops of the bowel in the chest and a
paucity of gas in the abdomen.
 The diaphragmatic margin is absent,
 There is a mediastinal shift to the opposite side and
only a small portion of the lung may be seen on the
ipsilateral side.
Diagnosis left-sided CDH

• Radiograph in a male neonate shows the tip


(large arrow) of the nasogastric tube
positioned in the left hemithorax. Note the
marked apex leftward angulation of the
umbilical venous catheter (small arrow).
Clinical Picture
o Postnatally, the most severely affected
babies present with
Respiratory Distress
o cyanosis, tachypnoea and sternal
recession at birth.
o Other infants Grunting respirations
within minutes or hours after birth.
Physical examination reveals:
A scaphoid abdomen.

An increased antero-posterior diameter


of the thorax and mediastinal shift.

 Breath sounds are absent on the affected


side.
Initial Management
Goal: oxygenate, avoid barotrauma
Intubated: conventional mechanical
ventilation

NGT for decompression


Preoperative measures:

• O2 administration to decrease R.D. and


cyanosis.
• Stomach decompression.
• Adequate hydration.
Treatment
Immediate surgical repair is
indicated because of the
severe life threatening
respiratory distress
associated with the
condition.
Operation:

• The content are reduced, the sac is


removed if present.
• The defect is closed with non
absorbable sutures and if large can
use a mesh.
Operative approach
Post operative care:

• O2 tent
• Frequent change s in position with
pharyngeal aspiration.
• I.V. fluids till effective peristalsis returns.
3.Anomalies
of the
Stomach
and
Duodenum
Hypertrophic Pyloric
Stenosis
Is hypertrophy of the muscle surrounding the

pylorus that leads to narrowing of the pyloric

canal and makes it difficult for the stomach to

empty. It occurs mainly in male infants.


It occurs when there is
hypertrophy of the circular muscle of
the pylorus leads to constriction of the
pyloric canal and obstruction of the
gastric outlet
Pathophysiology:

 The circular muscle of the pylorus is grossly enlarged


as a result of both hypertrophy (increased size) and
hyperplasia (increased mass).
 This produces severe narrowing of the pyloric canal
between the stomach and the duodenum.
Consequently, the lumen at this point is partially
obstructed over a period of time, inflammation and
edema further reduce the size of the opening until
the partial obstruction may progress to complete
obstruction.
 Vomiting: usually start in 2nd to 3rd week
of life. And becomes forceful and
projectile.
 Hungry and irritable later become
lethargic.
 Dehydration.
 Malnutrition.
 Constipation.
 Loss of skin turgor.
1. Palpable olive like mass in right upper
quadrant.
2.Visible, peristaltic waves usually form
the left to right.
3.Radiographic or ultrasound revealed
hypertrophied pyloric muscle.
4.Narrow pyloric sphincter revealed in
barium swallow. (Positive string sign).
Therapeutic Management
Surgical relief of the
pyloric obstruction by
Pyloromyotomy
Anomalies
of the
Intestine
There is congenital absence of
parasympathetic ganglion nerve cells of
a part of intestine usually in the distal end
of the descending colon.
Vary according to:
• Age when symptoms are recognized.
• The length of the affected bowel.
• Occurrence of complication, i.e.,
entrocolitis.
In the newborn:

 The primary signs and symptoms are:


 Failure to pass meconium within 24 – 48
hrs after birth.
 Food refusal.
 Vomiting.
 Abdominal distention.
During infancy:

• Inadequate weight gain.


• Constipation.
• Abdominal distention.
• Explosive watery diarrhea.
• Episodes of diarrhea and vomiting.
• Bloody diarrhea, fever and severe lethargy
are ominous signs because they signify the
presence of enterocolitis
During childhood:

• Constipation.
• Passage of ribbon like, foul smelling stool.
• Abdominal distention.
• Fecal impaction recurs frequently.
• Poor appetite, poor growth
• In neonate: diagnosis is usually based on
clinical signs of intestinal obstruction and
failure to pass meconium.
• On examination, the rectum is empty of
feces, the internal sphincter is tight and
leakage of liquid stool.
• Barium enema and rectal biopsy.
• Non-invasive procedure may be used as
anorectal manometry.
Therapeutic Management:
Treatment is primarily surgical removal of
Aganglionic portions of the bowel in
order to permit normal bowel motility
and establish continence by improved
functioning of the internal anal
sphincter.
Acquired Intestinal Defect
Intussusception
It is an acquired type
of intestinal
obstruction. It is the
invagination or
telescoping of a
segment of intestine
into the segment
immediately distal to
it.
Incidence:

Intussusception is one of the most


frequent causes of intestinal obstructions
in children between the ages of 3 months
and 5 years. Half of the cases occur in
children younger than 1 year, and most of
the others occur in children during the
second year.
Etiology:

Exact cause of Intussusception is uncertain but there are


several theories as:
 Greater disparity between the size of the ileum and
ileocecal value in infants is believed to encourage
telescoping at this point.
 Distorted peristalsis due to change of diet (weaning).
 Enlarged lymph nodes along the G.I.T. that occur with
respiratory infections, cystic fibrosis, foreign bodies, G.I
polyps.
 Hyperperistalsis.
 Diarrhea, constipation.
 Mobile ileocecal junction.
The most common sites:

 Ileocecal value. (Ileocolic)


 Ileoileal.
 Colocolic.
• Sudden acute abdominal pain, which
becomes progressively more severe.
–Child screams and draws the knees
into the chest.
–Child is healthy and normal
between episodes of pain.
• Vomiting.
• Lethargy.
• Passage of red current jelly stool. (Stool
mixed with blood and mucus).

• Palpable sausage- shaped mass in upper


right quadrant.

• Empty right lower quadrant (dance sign).


• Anorexia, discomfort.

• Tender, distended abdomen.


• Eventually fever, perforation, peritonitis
and shock are serious complications of
Intussusception.
Therapeutic Management:
• The initial treatment of choice is non-
surgical hydrostatic reduction.
• Traditionally by barium enema:
• In this procedure, correction of the
invagination is carried out at the same
time as the diagnostic testing.
• The force exerted by the flowing barium is
usually sufficient to push the invaginated
portion of the bowel into its original
position.
• It is incomplete development or
absence of the anus in its normal
position in the perineum
Types of Imperforated Anus:

1-Anal Stenosis: Narrowing of the anorectal canal


that may occur at any point or extended its
entire length.
2-Imperforated Anal Membrane: A thin
membrane persists across the anal opening.
3-Anal Agenesis: The rectum ends in a blind pouch
below (Low) or above (High) the levator ani
muscles.
4-Rectal Atresia: The anus is normal but the
rectal canal is not continuous. This type is very
rare
Assessment Criteria:

• Failure to pass meconium stool in the first


24 hours after birth.
• Absence or stenosis of the anorectal
canal.
• Anal membrane.
• External fistula to the perineum.
Diagnostic Procedures:

• During the initial assessment of the


newborn perineum, which should include
determination of the rectum patency with
small finger or rectal tube if a lesion is
suspected.
• X- ray examination.
• Ultrasound.
• Computed tomography scan.
Abdominal Wall Defects

 Omphalocele
It is herniation of intestines into the
umbilical cord, i.e., a protrusion, at birth,
of part of intestine through a defect in the
abdominal wall at the umbilicus.
Omphalocele

 There is a sac that covers the defect,


which composed of transparent
avascular membrane from which the
umbilical cord extends.
Gastrochisis
It is a congenital defect that differs from
omphalocele in that it is fissure of the
abdominal wall to the right of the
umbilicus and does not involve the site of
the insertion of the umbilical cord.
Protrusion of the small intestine and part of
large intestine usually accompany it.
There is no sac covering the intestine,
therefore, the bowel is irritated by
amniotic fluid during gestation and
becomes edematous and inflamed.
Umbilical Hernia

It is protrusion of the omentum and small intestine


through the imperfect closure or weak umbilical
ring, especially when infant cries.
Inguinal Hernia

It is protrusion of abdominal contents through the


inguinal canal into scrotum.
Nursing Management:
Pre-operative Care:
For cases of gastrochisis, omphalocele, umbilical and
inguinal hernias.
 Do not apply pressure on herniated area.
 Nasogastric suctioning for omphalocele / gastrochisis.
 Apply sterile dressing over herniated area for
omphalocele/ gastrochisis.
 Keep the child quiet, no crying in particular in cases of
harnias, especially umbilical hernia. Give warm bath,
suck pacifier to calm the infant.
 Educate parent about “ strape “ the defect has no value
to decrease hernia.
Post- operative Care:
Routine post-operative care, in addition to:
 For omphalocele and gastrochisis, the infant is fed by
peripheral Hyperalimentation
 Omphalocele infant then fed after that through
gastrostomy (increase amount of glucose water, then
milk).
For all cases :
 Observe are of defect for signs of infection.
 Maintain clear airway.
 Change position to prevent atelectasis.
 Emotional support for parents, especially in case of
omphalocele where the operation is done in steps.
Defect of Respiratory System
1. Choanal Atresia:
 It is defined as a congenital obstruction of the posterior
nares at the entrance to the naso-pharynx.
 It may be unilateral or bilateral
 Etiology:
The obstruction is usually caused by a
membrane, but in rare cases by a boney
growth.
 Assessment
In bilateral obstruction there is mouth
breathing and difficulty in taking feedings.
There may be dyspnea because the infant
can’t obtain enough oxygen.
Treatment
 It should be treated as early as possible
since it is one cause of asphyxia of the
newborn.
 If the obstruction is unilateral, the infant
may do well unless infection occurs and
persist on the side opposite the
obstruction.
2. Diaphragmatic Hernia
• It is a protrusion of the abdominal viscera, mainly the
intestine, through a defect in the diaphragm into the
chest cavity. It usually occurs in the left side of the chest.
The infant has large chest in comparison with his small
abdomen.
• Assessment
The infant usually develops respiratory
distress and possibly cyanosis.
• Treatment
Surgery. If operation is not done
immediately, the prognosis is very poor
and the patient will probably die within the
first month of life.
Spina Bifida:
It is a defective closure of the vertebral
column. Whether the defect will have
devastating consequences for the
neonates depends entirely on the site
and the extent of the anomaly.
Spina Bifida Occulta

• Usually the 5th lumber and 1st sacral


vertebrae are affected with no protrusion
of interspinal contents the spinal cord
and its cover the skin over the defect
may reveal a dimple, small fatty mass, or
a tuft of hair. The defect is only in the
vertebra, the spinal cord and meneiges is
normal and not visible.
Meningocle

• It is a protrusion through the spinal bifida,


which forms a soft, saclike appearance along
the spinal axis and contains spinal fluid and
meninges within the sac and covered with skin.
Meningomyelocele

• It is a more serious defect in which the


spinal cord and /or nerve roots as well as
meningoel covering protrude through the
spina bifida. The degree and extent of
neurogenice defect depend on the level
of the defect. The higher the level of the
defect, the greater the defect.
• If in the lumbosacral
area, the defect is
usually associated
with a flaccid
paralysis of the lower
extremities, absent
sensation to the level
of the lesion, and loss
of bowel and bladder
control.
Hydrocephalus

• The abnormal increase in cerebrospinal fluid volume


within the intracranial cavity due to a defect in the
cerebrospinal fluid drainage system, intracranial
pressure increases, the scalp veins dilate, and the
cranial structure begin to separate
Nursing Management of Meningocele and
Meningomyelocele
Nursing Diagnosis:
• High risk for infection.
• High risk for injury.
• High risk for skin impaired integrity.
• Altered nutrition: Less than body
requirements
Epispadias
Mieatual opening located on dorsal or
superior surface of the penis.
Hypospedias
Urethral opening located behind glands
penis or anywhere along ventral (lower)
surface of penile shaft.
N .B.: infants with epispadias and
hypospadias should not be circumcised
before repair of the defect because the
surgeon may wish to use apportion of
the foreskin for plastic repair.
3-Phimosis
Narrowing or stenosis of
preputial opening of
foreskin. In Severe
cases, circumcision or
vertical division and
transverse, suturing of
foreskin.
4-Hydrocele
Fluid in scrotum.
Therapeutic management
is surgical repair
indicated if spontaneous
resolution not
accomplished in 1 year.
5-Polycystic Kidney:

The infant has enlarged kidneys filled with


cysts at birth. If the condition is bilateral
the infant will not pass urine but if it is
unilateral the condition may be missed until
later in life.
6-Wilm’s Tumor (Ebryoma)
It is a malignant tumor of the kidney that arises
from an embryonic structure present in the child
before birth; the tumor is felt as an abdominal
mass. It is important that the necessary for
diagnosis because handling appears to increase
the danger of metastasis.
Clubfoot
Flexion at the ankle with inversion of
the heel and fore foot.
Torticollis
It is a condition in which there is a lateral
inclination and a rotation of the head
away from the midline of the body with
limitation of the range of motion of the
neck.
Congenital Dislocation of the Hip
In this condition the femur head is
completely dislocated from the
acetabulum. The infant shows limited
ability to abduct the hip, asymmetry of
the gluteal skin folds and inguinal
creases, and shortening of the affected
leg
Before cast application
★ Child should be allowed to play with a doll that has a
cast so that he understands what will be done.
★ The nurse must set up the cast materials and hold the
extremity in alignment.
★ Before the cast is applied, the extremities are checked
for any abrasions, cuts, or other alterations in skin
surface and other items that might cause constriction
from swelling; such objects are removed.
★ Identification bands are placed on a non-injured
extremity if hospitalization is anticipated.
After cast application
♥The cast must remain uncovered to allow is
dryness from inside out.
♥After the cast is applied, the extremities are
checked for any sings of compromise.
♥Observations such as pain, swelling,
discoloration (pallor or cyanosis) of the
exposes portions, lack of pulsation and
warmth, or the inability to move the exposed
parts are reported immediately.
♥ Turning the child in a plaster cast at least
every 2 hours to prevent complications from
immobility.
♥ The body part can be elevated, thereby
increasing venous return.
♥ Periodically the circumscribed blood stained
area should be outlined with a ball point pen
to assess the amount of blood.
♥ Parents need instructions on drying and
caring for the cast and checking for signs
that indicate the cast is too tight.