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Congenital Gastrointestinal Disorder

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Congenital Gastrointestinal Disorder puts the patient at risk for

aspiration
1. Ankyloglossia ( Tongue Tied) - allow the infant to breastfeed in an
- Is an abnormal restriction of the upright position
tongue caused by an abnormally - ensure the infant is bubbled well
tight frenulum after feeding because it has a
- Frenulum is the membrane tendency of swallowing too much
attached on the lower anterior tip air
of the tongue - if bottle feeding, use a commercial
- It can cause destructive damage on cleft palate nipple with extra flange
the gingival tissue and it can also to cover the root of the mouth
cause speech difficulty - if surgery is delayed up to 6 months,
- Can be managed by surgical release instruct the parent to give soft diet.
of the frenulum
2. Cleft lip and Palate Post operative management
- The fusion of the maxillary and - put the patient on NPO status for
median nasal sinuses occurs approximately 4hrs
between the 5th and 8th week of - introduce plain water after 4hrs
intrauterine life
- Failure in closure leads to the cleft 3. Tracheoesophageal Atresia and Fistula
lip
- It may caused by the exposure of
the mother to teratogens and virus
during the 5th and 8th week Assessment
- The palatal process closes at - Hydramnios
approximately 9th to 12th weeks of - Preterm
intrauterine life - Vertebral, Anal, Cardiac,
- A CLEFT PALATE is an opening of Tracheoesophageal, Renal, Limb
the palate, is usually on the midline (VACTERAL)
and may just involve the anterior - Coughing when breastfeeding
hard palate, posterior soft palate, or - Difficulty breathing and cyanosis
both - So much mucous in the mouth
- IT OCCURS MORE FREQUENTLY IN - Absent gastric aspirate
GIRLS
Management Management
- Early surgical management to allow - NPO
the infant enjoy the pleasure of - Upright position
sucking as early as possible - Gastronomy
- Early repair helps in the bonding - IV therapy
with the parents - Surgery closing the fistula and
Pre operative management anastomosis of segments
- Cleft lip and palate interferes with
the maintenance of suction and it
4. Gastroschisis - Intense crying and pain
- Protrusion of the abdominal - Pulling up the legs
content through an opening at the - Abdominal distension
abdominal wall so that abdominal - Vomiting
organs spill free out the abdomen
- The abdominal contents or not Management
contained in a peritonium - Surgical release of intestinal
- The abdominal wall defects is a twisting before the intestine get
distance from the umbilical organs necrosed
- NPO while the patient is not yet
Management undergone operation
- Support ABG
- NPO until the defect is repaired • INTUSSUCEPTION
- Insert OGT/NGT to avoid distension - It is the invagination of one portion
of intestines of the intestine into another
- Prevent drying of the organs
- Prevent infection Signs and symptoms
- TPN postoperatively - Suddenly draws up their legs and
cry as if they are in severe pain and
possibly vomit
5. Omphalocele - The vomits may contain bile
- A protrusion of the abdominal - Present blood in the stool
contents through the abdominal described as having “ current jelly
wall at the point of junction of the appearance”
umbilical cord and abdomen - If necrosis occurred
- The herniated organs are usually Elevated temp
intestines, but they may include Peritoneal irritation
liver or stomach Increased pulse and WBC
- The herniated is usually covered CONFIRMED BY SONOGRAM
with a thin membrane known as
peritonium Management
- Requires surgical intervention or
MANAGEMENT same with reduction by instillation of water
Gastroschisis soluble solution, barium enema or
air
6. Intestinal Obstruction - Same management as Volvulus
• VOLVULUS
- Is a twisting of the intestine. The 7. Diaphragmatic Hernia
twist leads to obstruction of the - Protrusion of an abdominal organ
passage of feces and compromise of through a defect in diaphragm into
the blood supply to the loop of the chest cavity
intestine involed - Usually occurs on the left side
causing cardiac displacement to the
Signs and symptoms
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
Microcephaly
Neural Tube Defects © Brain is smaller than normal
© Spina bifida- collective term for all spinal © May be associated with intrauterine
cord disorders infection or severe malnutrition during
• - literally means divided spine pregnancy
Caused by polygenic inheritance and © The infant is usually cognitively
bad nutrition during pregnancy challenged
• Low in folic acid (ideal is 4mg/day) © 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
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 © Management
© If you are a nurse, How could you take 1. Protect the area
care of this child? 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 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
Encephalocele
o Cranial meningocele or
myelomeningocele
© The defect occurs most commonly at the • Preop
occipital area but may occur as nasal or • Place a wet sterile gauze on the lesion to
nasopharyngeal defect prevent drying
o Generally covered by skin but can-also • Change diapers frequently to prevent
be covered only by dura prolonged contact with urine and feces
ASSESSMENT • Observe for any leak of clear fluid from the
© Can be discovered while in uterus through lesion
sonography, fetoscopy, amniocentesis • Give family some support
(discovery of increased AFP in the amniotic
fluid), or analysis of AFP in the maternal
serum
© Defects can by easily observed at birth o Postop
• Compare body circumferences • Position the child on the unaffected side and
© Assess for spontaneous movement of avoid contact of the operative site with urine
extremeties and
© Asses for pattern of voiding and defecation 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

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