Cleft Lip/Palate
apparatus applied before surgery to
 the fusion fails to occur in varying
degrees, causing this disorder to range
from a small notch in the upper lip to
total separation of the lip and facial
structures up into the floor of the nose,
with even the upper teeth and gingiva
absent.
Cleft lip (Cheiloschisis)
 is more prevalent among boys than girls
and occurs at a rate of approximately 1
in every 700 live births
 It appears to be caused by the
transmission of multiple genes aided by
teratogenic factors present during weeks
5 to 8 of intrauterine life, such as a viral
infection, certain seizure medicines such
as phenytoin, maternal smoking or binge
drinking, hyperthermia, stress, and
maternal obesity.
 Folic acid deficiency may also be
associated with incomplete anterior
midline closures
Cleft palate (Palatoschisis)
 an opening of the palate, is usually on
the midline and may involve the anterior
hard palate, the posterior soft palate, or
both o It may be a separate anomaly, but
as a rule it occurs in conjunction with
cleft lip o It tends to occur more
frequently in girls than boys.
 May need caring support to bond with
an infant whose face is deformed in this
way
 Facial contours change as a child grows,
a revision of the original repair or a nasal
rhinoplasty to straighten a deviated
nasal septum may be necessary when
the child reaches 4 to 6 years of age.
 Some infants may have a nasal mold
shape a better nostrils.
Assessment
 Sonogram
 Inspection of the mouth at birth.
 Assess the child for other congenital
anomalies
Therapeutic Management
 Cleft lip is repaired surgically shortly
after birth, sometimes at the time of the
initial hospital stay or between 2 and 10
weeks of age
 A revision of the original repair may be
necessary when the child reaches 4 to 6
years of age
 The repair of cleft palate is usually
postponed until a child is 6 to 18 months
old.
 Repairs made before this change may be
ineffective and may have to be repeated
 Early repair also helps infants experience
the pleasure of sucking
Preoperative Period
 It may be possible for an infant with a
cleft lip to breastfeed because the bulk
of the mother's breast tends to form a
seal against the incomplete upper lip
 The best feeding method for the child
with cleft lip may be to support the baby
in an upright position and feed the infant
gently using a commercial cleft lip nipple
 If the surgical repair will be done
immediately, the mother will be able to
breastfeed as early as 7 to 10 days after
surgery
 Be certain an infant with a cleft lip is
bubbled well after feeding because of a
tendency to swallow air caused by the
inability to grasp a nipple or syringe edge
securely with the mouth
 If a cleft extends to the nares, an infant
will breathe through the mouth, causing
the oral mucous membranes and lips to
become dry
 Offering small sips of fluid between
feedings can help keep the mucous
membranes moist and prevent cracks
and fissures that could lead to infection
 Infants with cleft palate cannot suck
effectively either, because pressing their
tongue or a nipple against the roof of
their mouth could force milk up into
their pharynx, leading to aspiration
 The most successful method for feeding
this infant, like the child with cleft lip, is
to use a commercial cleft palate nipple
that has an extra flange of rubber to
close the roof of the mouth
Preoperative Period
 A Breck feeder, am apparatus similar to a
bulb syringe, or a Haberman feeder may
be used
 The nipple can be used with a plastic
bottle that can be squeezed gently to
increase the flow of the feeding to
compensate for poor sucking
Postoperative Period
 After surgery for cleft lip or palate, an
infant is kept NO for approximately 4
hours
 The infant is then introduced to small
amount of liquids (plain water) to
prevent vomiting
 No tension is placed on a lip suture line
to keep the sutures from falling apart
and leaving a large scar
 The infant is usually feed using a
specialized feeder because this causes
less suture line tension than bottle or
breastfeeding After Palate Surgery
 Liquids are generally continued for the
first 3 to 4 days and then a soft diet is
folled until healing is complete
 When the child begin eating soft food he
should not use a spoon because a child
will invariably put it against the roof of
the mouth and possibly disrupts sutures
 Be certain milk is not included in the first
fluids offered because milk curd tend to
adhere to the suture line
 After feeding offer the child clear water
to rinse the suture line and keep is as
clean as possible
Imperforate Anus
 is a stricture or the absence of the anus,
 In week 7 of intrauterine life, the upper
bowel elongates to pouch and combine
with a pouch invaginating from the
perineum.
 These two sections of bowel meet, the
membranes between them are
absorbed, and the bowel is then patent
to the outside.
 If this motion toward each other does
not occur or if the membrane between
the two surfaces does not dissolve, an
imperforate anus occurs.
Assessment
 The condition may be detected by a
prenatal sonogram.
 It is discovered at birth when inspection
of a newborn's anal region
 A "wink" reflex (touching the skin near
the rectum should make the anus
contract) cannot be elicited if sensory
nerve endings in the rectum are not
intact.
 Even with all these methods, some
instances of the stricture will not be
detected at birth because the anus
 appears as usual and the stricture exists
so far inside that it can't be seen.
 By 24 hours, no stool will be passed, and
abdominal distention will become
evident.
 An Xray or sonogram will reveal the
disorder if the infant is held in a slightly
head down position to allow swallowed
air to rise to the end of the blind pouch
of the bowel.
 This method is also helpful to estimate
the distance the intestine is separated
from the perineum or the extent of the
correction that will be necessary.
Therapeutic Management
 The degree of difficulty in repairing an
imperforate anus depends on the extent
of the problem.
 If the rectum ends close to the perineum
(at or below the level of the levator ant
muscle) and the anal sphincter is
formed, repair involves simple
laparoscopy with anastomosis of the
separated bowel segments.
 A repair becomes complicated if the end
of the rectum is at a distance from the
perineum (above the levator ani muscle),
the anal sphincter exists only in an
underdeveloped form, or a fistula to the
bladder or vagina is present.
 If the repair is estimated to be extensive,
the child may be given a temporary
colostomy and the final repair
performed when the infant is somewhat
older (6 to 12 months)
HIRSHSPRUNG'S DISEASE
 is a disorder of the gut caused due to
congenital absence of ganglion cells in
the submucosal and myentric plexus of
intestine. It is also known as Megacolon
or Congenital Aganglionic Megacolon.
Infants:
• Failure to pass meconium
• Ribbonlike stools (stools passing through
such a small, narrow segment look like
ribbons)
• Abdominal distension
• Bile- stained vomiting
• Shock
• Episodes of diarrhea & constipation
In older children:
• Constipation with abdominal distension
• When stool passed foul smelling, and liquid
in consistency
• Malnourished and anemic
DIAGNOSTIC EVALUATION:
• Hirschsprung's disease is suspected in a
baby who has not passed meconium within
48 hours of birth.
•Rectal examination
•Palpation
•Anorectal manometry
•Barium enema & Rectal biopsy
Therapeutic Management
 Repair of aganglionic megacolon involves
dissection and removal of the affected
section, with anastomosis of the
intestine (termed a pull through
operation).
 Because this is a technically difficult
operation to perform in a small
abdomen, the condition is generally
treated in infants by two stage surgery o
first, a temporary colostomy is
established, followed by bowel repair at
12 to 18 months of age. o After the final
 surgery, children should have a Post-operative care:
functioning, normal bowel.
I. Monitor vital signs, observe
 In the few instances in which the anus is
abdominal bleeding.
deprived of nerve endings, a permanent
II. Place the child in comfortable
colostomy will need to be established
position according to the physician
Repair of aganglionic megacolon involves
order.
dissection and removal of the affected
III. Child is NPO, so administer IV fluids
section, with anastomosis of the
as ordered
intestine (termed a pull through
IV. Monitor bowel sound.
operation).
V. Colostomy care is to be done which
 Surgeons frequently perform a single
includes following:
operation to fix intestinal obstruction
 Observe stoma for its colour (reddish-
when Hirschsprungs disease is initially
pink colour)
diagnosed. The goal of the surgery is to
 Observe for bleeding, purulent drainage,
remove the diseased section of the
edema.
intestine and to pull the healthy portion
 Provide bland diet
of the intestine down to the anus. This is
 apply zinc oxide ointment on skin
called a pull-through procedure. In most
around stoma.
cases, this surgery can be done with
minimally invasive techniques. It can Frequently empty the collecting bag
sometimes be performed entirely
through the anus, leaving no scars at all. 1. Keep the colostomy clean and dry.
2. Educate parents about colostomy
NURSING MANAGEMENT: care.
3. Encouraging and supporting the
Pre- operative care:
family during this stressful time is the
I. Assessment, complete history of key nursing intervention.
new born
II. After diagnosis, nurse must help
parents.
III. Nurse taught about giving isotonic
enema, suppositories and stool
softeners.
IV. Low residue diet must be given to
the child.
V. Monitor vital signs & abdominal
girth of child
VI. Keep the child in semi-fowlers
position.
VII. Withhold oral feeds & Nasogastric
aspiration done on the night before
surgery.
The history of medical research on human
subject is as old as the history of medicine
Itself. Ancient physicians believed that
dissection of human cadaver to study human
anatomy was insufficient to gain knowledge
about the malfunctions of the body.
 Vivisection - a form of ancient
experimentation performed on living
animals including man, using operation
(cutting or dissecting) designed to
promote some knowledge of
physiological and pathological processes.
 The Nuremberg Code, (the birth of
Informed consent) a 10 point principles
lo be fallowed in human
experimentation and foremost of these
is voluntary and Informed consent of
subject
subject against even the remote
possibilities of injury, disability or
death.
8. It should be conducted only by
scientifically qualified persons with
highest degree of skill throughout
the experiment.
9. During experiment the human
subject is free to discontinue the
process should he feel physically and
mentally incapable to continue.
10. Like-wise the scientist in charge of
the experiment must be prepared to
terminate it if there is probable cause
that the experiment might result in
injury or death to experimental
subject.
Justification for human experimentation:

Nuremberg 10point principles: 1. Animal studies are Inadequate

because human physiology and
1. The voluntary consent of subject is
psychology are different and unique.
absolutely essential.
2. The biochemical constitution and
2. The experiment should yield a fruitful
metabolism of humans are very
result for the good of society. cannot
much different from those of
be achieved by other methods, not
animals.
random and unnecessary
3. Contemporary medicines have
3. Should be designed based on result
become effective because il has
of animal experimentation and the
become scientific.
justified results will justly the
4. Experimentation on human subjects
performance of the experiment.
is necessary if we have to develop
4. It should be so conducted as to avoid
new drugs, new therapies, and new
all unnecessary physical. mental
methods of prevention for serious
suffering and injury
diseases that beset mankind.
5. No experiment should be conducted
5. It is necessary to develop certain
if death or disabling injury might
vaccines and drugs that can stop the
occur except it the physician will
spread of contagious diseases.
serve as subject.
6. The degree of risk should never
The moral issue of human
exceed by the humanitarian
experimentation is not to help o
importance of the problem to be
experimental subject but to advance
solved.
future knowledge for future patients.
7. Proper preparation and adequate
facilities be provided to protect
Two kinds of experiments: 5. Answer any questions asked about
the procedure
a) Therapeutic
6. Make It clear that he is free to
b) Non-therapeutic
withdraw consent w/out penalty

Informed consent is also

Medical therapy (therapeutic experiment) -
Is designed and conducted for the benefits of
the experimental subject either to diagnose
or treat an illness.
Purposes
1. Recognition of individual's
autonomy
2. The right to sell-determination
3. To form his own opinion

1. to relieve suffering and to restore to

health
2. To cure diseases
3. correct disorders
4. bring about normal bodily
functioning

Medical research (non-therapeutic

experiments) not designed to benefit the
experimental subject directly but only to
derive knowledge that can be utilized in the
treatment of other persons with similar
aliments.

Purposes:

a) To acquire better understanding of

the chemical and physiological
processes that are involved in
human functioning
b) Is Interested in the effectiveness of
therapies in ending disease
processes and restoring functioning
c) Interested in the formulation of
theories that can serve as a basis for
treating other individuals

Information element of informed consent:

1. explain procedure and purposes

2. Underscore risk and discomfort
expected
3. Describe possible benefits of the
experiment
4. Point out appropriate alternatives