Congenital Gastrointestinal Disorder
aspiration
1. Ankyloglossia ( Tongue Tied)
- Is an abnormal restriction of the
tongue caused by an abnormally
tight frenulum
- Frenulum is the membrane
attached on the lower anterior tip
of the tongue
- It can cause destructive damage on
the gingival tissue and it can also
cause speech difficulty
- Can be managed by surgical release
of the frenulum
2. Cleft lip and Palate
- The fusion of the maxillary and
median nasal sinuses occurs
between the 5th and 8th week of
intrauterine life
- Failure in closure leads to the cleft
lip
- It may caused by the exposure of
the mother to teratogens and virus
during the 5th and 8th week
- The palatal process closes at
approximately 9th to 12th weeks of
intrauterine life
- A CLEFT PALATE is an opening of
the palate, is usually on the midline
and may just involve the anterior
hard palate, posterior soft palate, or
both
- IT OCCURS MORE FREQUENTLY IN
GIRLS
Management
- Early surgical management to allow
the infant enjoy the pleasure of
sucking as early as possible
- Early repair helps in the bonding
with the parents
Pre operative management
- Cleft lip and palate interferes with
the maintenance of suction and it
puts the patient at risk for
- allow the infant to breastfeed in an
upright position
- ensure the infant is bubbled well
after feeding because it has a
tendency of swallowing too much
air
- if bottle feeding, use a commercial
cleft palate nipple with extra flange
to cover the root of the mouth
- if surgery is delayed up to 6 months,
instruct the parent to give soft diet.
Post operative management
- put the patient on NPO status for
approximately 4hrs
- introduce plain water after 4hrs
3. Tracheoesophageal Atresia and Fistula
Assessment
- Hydramnios
- Preterm
- Vertebral, Anal, Cardiac,
Tracheoesophageal, Renal, Limb
(VACTERAL)
- Coughing when breastfeeding
- Difficulty breathing and cyanosis
- So much mucous in the mouth
- Absent gastric aspirate
Management
- NPO
- Upright position
- Gastronomy
- IV therapy
- Surgery closing the fistula and
anastomosis of segments
 4. Gastroschisis
- Protrusion of the abdominal
content through an opening at the
abdominal wall so that abdominal
organs spill free out the abdomen
- The abdominal contents or not
contained in a peritonium
- The abdominal wall defects is a
distance from the umbilical organs
Management
- Support ABG
- NPO until the defect is repaired
- Insert OGT/NGT to avoid distension
of intestines
- Prevent drying of the organs
- Prevent infection
- TPN postoperatively
5. Omphalocele
- A protrusion of the abdominal
contents through the abdominal
wall at the point of junction of the
umbilical cord and abdomen
- The herniated organs are usually
intestines, but they may include
liver or stomach
- The herniated is usually covered
with a thin membrane known as
peritonium
MANAGEMENT same with
Gastroschisis
6. Intestinal Obstruction
• VOLVULUS
- Is a twisting of the intestine. The
twist leads to obstruction of the
passage of feces and compromise of
the blood supply to the loop of
intestine involed
Signs and symptoms
- Intense crying and pain
- Pulling up the legs
- Abdominal distension
- Vomiting
Management
- Surgical release of intestinal
twisting before the intestine get
necrosed
- NPO while the patient is not yet
undergone operation
• INTUSSUCEPTION
- It is the invagination of one portion
of the intestine into another
Signs and symptoms
- Suddenly draws up their legs and
cry as if they are in severe pain and
possibly vomit
- The vomits may contain bile
- Present blood in the stool
described as having “ current jelly
appearance”
- If necrosis occurred
Elevated temp
Peritoneal irritation
Increased pulse and WBC
CONFIRMED BY SONOGRAM
Management
- Requires surgical intervention or
reduction by instillation of water
soluble solution, barium enema or
air
- Same management as Volvulus
7. Diaphragmatic Hernia
- Protrusion of an abdominal organ
through a defect in diaphragm into
the chest cavity
- Usually occurs on the left side
causing cardiac displacement to the
 right side of the chest and collapse 9. Meconium Plug Syndrome
the left lung • Is an extremely hard portion of meconium
that has
Signs and symptoms completely blocked the intestinal lumen,
- Respiratory difficulty from the time causing
of birth bowel obstruction.
- Cyanosis, intercostal retractions • Presents with abdominal distension, emesis
- Abdominal circumference lower failure to pass meconium within the first 24 to
than normal 48
- Sunken abdomen hours of life.
- Absence of breath sound at the • Associated with Hirschsprung disease, cystic
affected part fibrosis,
hypothyroidism and magnesium sulfate to halt
Management preterm labor
- Emergency surgical repair of the
diaphragm before surgery: Assessment
Position the patient with head • Abdominal distension and vomiting after 24 to
elevated 48
Insert NGT hours after delivery
Keep patient on NPO • Rectal exam may show hardened stool
- After surgery • Sonogram shows air-filled loops in the stool
Put patient in fowlers position
Keep in a warm humidified Management
environment Barium enema
Acetylcysteine rectal suppository
8. Diaphramatic Hernia Umbilical Hernia
Signs and symptoms • A protrusion of a portion of the intestine
• Respiratory difficulty from the time of birth through the
• Cyanosis, intercostal retractions umbilical ring, muscle, fascia surrounding the
• Abdominal circumference lower than normal umbilical cord
• Sunken abdomen • There is a bulging protrusion under the skin at
• Absence of breathsound at the affected part the
umbilicus
Management • The bulging increases as the child restrains or
• Emergency surgical repair of the diaphragm cry
before surgery: • If the bulge < 2cm, it will close simultaneously
• Position the patient with head elevated • If the bulge > 2cm, surgical closure is needed
• Insert NGT • Postop- put pressure dressing on the surgical
• Keep patient on NPO site
After surgery • Sponge bath till the next visit
• Put patient in Fowler's position
• Keep in warm humidified environment 10. Imperforate anus
• Stricture of the anus
• More common in boys
Imperforate anus • Distention of the portion of the bowel
Assessment proximal to the
• Assess temperature rectally affected area distends leading to abdominal
• No anus present on inspection distention
• Absence of meconium • No meconium passed after 24 hours
• Abdominal distention • No feces at the terminal end of bowel on
• Check for presence of meconium in the urine digital exam
• Management
• Surgical repair of the anus or formation of • Dissection of the affected section and removal
temporary of the affected section with anastomosis of the
colostomy that will be closed in 6mo to 1 yr intestine
• Preop- keep on NPO to avoid farther bowel • It involves the formation of temporary
distension colostomy followed by repair at 12 to 18 months
- insert NGT and connect to low suction
- insert IV line • Preop- daily NSS enema
• Postop-NGT still inserted until bowel sound is • (2 tsp non iodized salt 1 quart water)
assessed • Low residue diet
- small frequent feedings with glucose water, • Stool softener daily
formula or • Postop- insert NGT in low suction
breastmilk • Observe for abdominal distention
• Axillary or tympanic temperature after rectal • Assess bowel sound
repair • Small frequent feeding of fluid once NGT is
• No suppositories or enema removed
• Stool softeners daily
• If colostomy is formed, give low residue diet
• Postop- clean suture line every after bowel
movement
• Place diaper under, not on the infant
• Side lying position
• If colostomy is formed, teach the parents on
colostomy care

11. Hirschprung's disease (Aganglionic

megacolon)
• Absence of ganglionic inervation to the muscle
of a
section of the bowel, most commonly the
sigmoid
colon
• No peristaltic movement at this section of the
colon
• No movement of feces at this area leading to
chronic
constipation or passing of ribbon-like stool
Neurologic development disorders
Neural Tube Defects
© Spina bifida- collective term for all spinal
cord disorders
• - literally means divided spine
Caused by polygenic inheritance and
bad nutrition during pregnancy
• Low in folic acid (ideal is 4mg/day)
Anencephaly
© Absence of cerebral hemispheres
© Upper end of neural tube fails to close at
an early intrauterine life
© THE CHILD IS LIKELY TO DIE
© The purpose of its delivery may be to
donate its organ to another patient
needing it
© If you are a nurse, How could you take
care of this child?
Microcephaly
© Brain is smaller than normal
© May be associated with intrauterine
infection or severe malnutrition during
pregnancy
© The infant is usually cognitively
challenged
© Must be distinguished from
craniosynestosis because they both
cause smaller brain than normal
Spina Bifida Occulta
© The posterior laminae of the vertebrae
fails to fuse
© There may be dimpling at the point of
poor fusion or there may be tufts of hair
at the area.
© It is a benign defect
Meningocele
© The meninges covering the spinal cord
and the meninges herniate through an
unformed vertebrae
• The anomaly appears as a protruding
mass usually approximately the size of
an orange at the center of the back
• The protrusion may be covered by skin
or only the clear dura mater
© Management
1. Protect the area
1. Put the client in prone or side lying position
2. If the area is covered by dura mater,
prevent drying and infection
Myelomeningocoel
© Spinal cord and meninges protrude
through an unformed vertebrae.
© Spinal cord ends at the region of the
defect, therefore there are no motor and
sensory nerves beyond these points
© loss of sensory, flacidity, loss of bowel
Control
Encephalocele
o Cranial meningocele or
myelomeningocele
© The defect occurs most commonly at the
occipital area but may occur as nasal or
nasopharyngeal defect
o Generally covered by skin but can-also
be covered only by dura
ASSESSMENT
© Can be discovered while in uterus through
sonography, fetoscopy, amniocentesis
(discovery of increased AFP in the amniotic
fluid), or analysis of AFP in the maternal
serum
© Defects can by easily observed at birth
• Compare body circumferences
© Assess for spontaneous movement of
extremeties
© Asses for pattern of voiding and defecation
Management
• Once discovered in uterus, CS is done
© Perform routine perioperative management
• Prevent drying and infection while waiting for
surgery
® For meningocele, myelomeningocele, and
encephalocele- surgery to replace the
contents that are irreplaceable and to close
the skin defect to prevent infection
• Preop
• Place a wet sterile gauze on the lesion to
prevent drying
• Change diapers frequently to prevent
prolonged contact with urine and feces
• Observe for any leak of clear fluid from the
lesion
• Give family some support
o Postop
• Position the child on the unaffected side and
avoid contact of the operative site with urine
and
feces
• Assess for increased ICP
• Feed the child in a normal position as possible
and avoid pressing the operative site
• When bubbling, avoid tapping at the operative
site
• Give non-nutritive diet
• Role model a warm, comforting parent
© Before discharge
• Provide passive ROM to prevent
contractures and atrophy of paralyzed body
part; teach parents on how to perform this
procedure
• Assess paralyzed body parts to detect skin
breakdown or any damage
• When child is using wheel chair, teach to
press their arms on the armrest to raise the
buttocks
• Teach intermittent catheterization technique