Cleft Lip or Cleft Palate • In bilateral cleft lip, the midportion of the

upper lip is attached on both sides and may

➢ A commonly occurring congenital midline
be displaced forward.
fissure, or opening, in the lip or palate
• Dental anomalies (e.g., missing,
resulting from failure of the primary palate to
mispositioned, or deformed teeth) are
fuse
common.
➢ Results from failure of the maxillary and
premaxillary processes to fuse during the 5th Cleft Palate
to 8th week of intrauterine life
➢ Occurs when the primary and the secondary
Causes: palatine plates fail to fuse during the
embryonic development
1. Genetic and environmental factors
➢ May involve only the soft palate or may
2. Family history (strongest risk factor)
extend into the hard palate
3. Maternal smoking, consumption of large
➢ May occur only in the midline of the posterior
amounts of alcohol, medications taken during
palate or may extend to the nostril on one or
the first trimester, and maternal DM (not
both sides
gestational diabetes)
Complications:
Development (intrauterine)
1. Difficulty feeding. While most babies with
1. During the 4th week of development, a series
cleft lip can breastfeed, a cleft palate may
of six paired (right and left) swellings form
make sucking difficult.
near the cranial end of the fetus. These are
2. Dental problems. If the cleft extends to the
called brachial arches.
upper gum, tooth development may be
2. The first brachial arche develop structures
affected.
called the maxillary and the mandibular
3. Speech difficulties. Because the palate is
prominences.
used in forming sounds, the development of
3. In the center of the upper face, there is a
normal speech can be affected by cleft palate.
structure called the frontonasal prominence.
Speech may sound too nasal.
Cleft Lip 4. Challenges of coping with a medical
condition. Children with clefts may face
➢ Results from embryonic fusion of the
social, emotional, and behavioral problems
embryonic structures surrounding the
due to differences in appearance and the
primitive oral cavity
stress of intensive medical care.
➢ May be unilateral or bilateral
➢ Associated with abnormal development of Nursing Interventions:
the external nose, nasal cartilages, nasal
1. Maintain adequate nutrition.
septum, and maxillary alveolar ridges
A soft nipple with a cross-cut made to
Note: promote easy flow of milk may work well.
Positioning
• The extent of the cleft varies, from an
The infant should be kept in an upright
indentation in the lip to a deep and wide
position during feeding.
fissure extending to the nostril
Tools for feeding.
• In severe clefts, the nostril on the affected Lamb’s nipples (extra long nipples) and
side is low and,the nose is deviated to that special cleft palate nipples molded to fit into
side the open palate area to close the gap may be
used.
 One of the most effective methods may be the
use of an eyedropper or an asepto syringe
with a short piece of rubber tubing on the tip
(Breck feeder).
2. Promote family coping
3. Reduce family anxiety
4. Provide family teaching
Hirschsprung’s Disease
(Congenital Aganglionic Megacolon)
• Characterized by persistent constipation
resulting from partial or complete intestinal
obstruction of mechanical origin.
• Normally, when a stool enters the rectum, the
internal sphincter relaxes and the stools are
evacuated. In HD, the internal sphincter does
not relax.
Pathophysiology
• Results from the absence of enteric neurons
within the mysenteric and submucosal plexus
of the rectum and/or colon.
• Enteric neurons are derived from the neural
crest and migrate caudally with the vagal
nerve fibers along the intestine
• These ganglion cells arrive in the proximal
colon by 8 weeks gestation and in the rectum
by 12 weeks gestation.
• Arrest in migration leads to an aganglionic
segment.
Clinical Manifestation
Abdominal distention. Infants with aganglionic
megacolon show tympanic abdominal distention and
symptoms of intestinal obstruction.
Chronic constipation. Older infants and children
with HD usually present with chronic constipation.
Palpable intestinal loop. Upon abdominal
examination, these children may demonstrate
marked abdominal distention with palpable dilated
loops of colon.
Absence/ delayed passage of meconium. During
the newborn period, infants affected with HD may
present failure of passage of meconium.
Vomiting. Repeated vomiting is present due to
intestinal obstruction.
Malnourishment. Poor nutrition results from the
early satiety, abdominal discomfort, and distention
associated with chronic constipation.
Fever and explosive diarrhea indicate enterocolitis,
a life-threatening condition.
Diagnostic Findings
1. Laboratory studies (CBC). Order test if
enterocolitis is suspected. Elevation of WBC
count should raise concern for enterocolitis.
2. Plain abdominal radiography. Perform this
test with any signs and symptoms of
abdominal obstruction.
3. Unprepared single-contrast barium
enema. If perforation and enterocolitis is not
suspected, this may help to establish the
diagnosis by identifying a transition zone
between a narrowed aganglionic segment and
a dilated and normally innervated segment.
The study may also reveal a non-distensible
rectum, which is a classic sign of HD.
4. Rectal biopsy. Diagnosis is confirmed
through rectal biopsy. It is a procedure used
to extract a tissue sample from the rectum for
a laboratory analysis. The rectum is the
lowest 6 inches of the large intestine located
just above the anal canal. The rectum’s
purpose is to store the body’s solid waste
until it is released.
Medical Management
Initial therapy. If a child with Hirschsprung’s
disease has s/sx of a high grade intestinal obstruction,
initial therapy should include intravenous hydration,
withholding of enteral intake, and intestinal and
gastric decompression.
Decompression. Decompression can be
accomplished through placement of a nasogastric
tube and either digital rectal examination or normal
saline rectal irrigations 3-4 times daily.
Diet. A special diet is not required. However,
preoperative and in the early post-operative period,
infants of a non-constipated regimen, such as
breastmilk, are more easily managed.
Surgical Management
1. Single stage pull-through procedure
May be performed with laparoscopic, open,
or transanal techniques. This procedure can
be performed at the time of diagnosis or after
the newborn has had rectal irrigation at home
and has passed the physiologic nadir.
2. Leveling colostomy.
A colostomy at the level of normal bowel. A
staged procedure with placement of a leveled
colostomy followed by a pull-through
procedure.
Nursing Management
Promote skin integrity. When performing routine
colostomy care, give careful attention to the area
around the colostomy. Record and report redness,
irritation, and rashy appearancs of the skin around the
stoma. Prepare the skin with skin-toughening
preparations that strengthen it and provide better
adhesion of the appliance.
Promote comfort. Observe for s/sx of pain, such as
crying, pulse, and respiration rate increases,
restlessness, guarding of the abdomen, or drawing up
the legs.
Administer analgesics as ordered.
Additional nursing intervention include: changing
the child’s position, holding the child when possible,
stroking, cuddling, and engaging in age-appropriate
activities.
Maintain fluid balance. Accurate intake and output
determinations and reporting the character, amount,
and consistency of stools help determine when the
child may have oral feedings. To monitor fluid loss,
record and report drainage from the NG tube every 8
hours, and immediately report any unusual drainage
such as bright-red bleeding.
Imperforate Anus
Clinical Manifestations
Absence of stools. There is no passage of stools
within a day or two after birth.
Passing of stools in other opening. The infant may
pass stools through another opening like the urethra
in boys or vagina in girls.
Swollen belly. The newborn could not pass stools,
resulting in a swollen belly.
Absence of anal opening. The opening of the anus
is missing or not in its usual place. In girls, this may
be near the vagina.
Diagnostic Findings
Sacral radiography. Two views of the sacrum,
posteroanterior and lateral, should be obtained to
measure sacral ratios and to look for sacral defects,
hemivertebrae, and presacral masses. This should be
performed before surgery.
Abdominal ultrasonography. This study is
specifically used to examine the GU tract and to look
for any other masses.
MRI. All children who have sacral defects on plain
radiographs should undergo spine ultrasonography to
rule out associated malformations such as
meningococele, teratoma, or mixed lesions.
CT scan. CT scanning presently plays no role in the
routine evaluation of children with anorectal
malformations.
Medical Management
1. Nothing per orem
2. Neonatal colostomy. A colostomy is
performed in children who are not responsive
to primary pull-through either because of
malformation.
3. Primary neonatal pull-through without
colostomy.