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Post R.N B.Sc Nursing


Khyber Medical University

Post Graduate College of Nursing Peshawar
 Birth defect is a widely-used term for a
congenital malformation, i.e. a congenital,
physical anomaly which is recognizable at birth,
and which is significant enough to be
considered a problem. According to the
(Centers for Disease Control and Prevention,
CDC) most birth defects are believed to be
caused by a complex mix of factors including
genetics, environment, and behaviors, though
many birth defects have no known cause.

AIM: To share the knowledge among the
participants about the Cogenital
disorders of G.I system.
At the end of presentation the participants will be able to:
 Describe the normal characteristics of pediatric G.I
 Define congenital disorder.
 Enlist the congenital disorders of G.I system.
 Explain different congenital abnormalities of G.I system
with causes, pathophysiology and signs & symptoms.
 Describe the nursing care plan for each disorder.

Characteristics of the
pediatric GI System
 Peristalsis occurs within 2 ½ to 3 hours in
the neonate and extends to 3 to 6 hours in
older infants and children.

 The stomach capacity of the neonate is 30

to 60 ml, which gradually increases to 200
to 350 ml by age 12 months and 1,500 ml
in the adolescent.

 Up until 4 to 8 weeks, the neonatal
abdomen is larger than the chest and the
musculature is poorly developed.

 The sucking and extrusion reflexes persist

until 3 to 4 months.

 Saliva production begins at age 4 months

and aids in the process of digestion.

 Spit-ups are frequent in the neonate because
of the immature muscle tone of the lower
esophageal sphincter (LES) and the low
volume capacity of the stomach.

 Increased myelination of nerves to the anal

sphincter allows for physiologic control of
bowel function, usually at about age of 2

 The liver’s slow development of
glycogen storage capacity makes the
infant prone to hypoglycemia.

 From ages 1 to 3 years, the composition

of intestinal flora becomes more
adultlike and stomach acidity increases,
reducing the number of GI infections.
What is congenital disorder?
 Congenital disorder involves defects in or damage
to a developing fetus. It may be the result of genetic
abnormalities, the intrauterine (uterus) environment,
errors of morphogenesis, or a chromosomal

 Any condition acquired during development in the

uterus and not through heredity.

 Describes a disease or condition with which someone

is born.
 8
Congenital G.I Disorders
 Cleft lip & cleft palate.
 Esophageal atresia or transesophageal fistula.
 Gastroesophageal Reflux.
 Omphalocele.
 Hirschprung’s Disease.
 Intussusception.
 Pyloric Stenosis.
 Diaphragmatic Hernia.
 Celiac Disease.
 Imparforate anus.

Cleft Lip
 Cleft lip and palate Tx: surgery –
results when fusion Cheiloplasty;
involving the first
brachial arch faills to to usually 1-3 mos
take place uring
embryonic Rule of 10’s-
devellopment. failure of 10 wks, 10 lbs,
fusion of maxillary and
median nasal process Hgb 10 gm/dl
 hereditary
 unilateral/bilateral
 males

Cleft Palate

 midline opening of palate

 usually with Cleft lip
 Female
 surgery 6-18 mos - allows
anatomic changes;
 (ie, formation of palatine
arch and tooth buds)

Pathophysiology of cleft lip &
 Cleft lip
 Varies from a notch in the lip to complete separation of the lip into the
 Failure of maxillary process to fuse with nasal elevations on frontal
prominence; normally occurs during 5th and 6th week of gestation.
 Merge of upper lip at midline complete b/w 7th and 8th week of

 Cleft palate
 Failure of mesodermal masses of lateral palatine process to meet and
fuse; normally occurs b/w 7th and 12th week of gestatioon.

• s/s: ?????

Nursing Dx:
 Risk for imbalanced nutrition, less than body
requirements R/T feeding problems

 Risk for ineffective airway clearance R/T oral surgery

 Risk for infection during post op period

Nursing Care
 Adequate nutrition; sips of fluid btw feeding in upright
position; use rubber tipped syringe; Burp
 Prevent ear and upper respiratory tract infection
 Address body image and speech concerns
 Reassurance to parents

 monitor respiratory distress d/t edema, hemorrhage
 Suction mucus and blood gently
 dropper feeding 1st 3 weeks; regular feeding after
 Anticipate needs and position to prevent tension on sutures

• Position side lying in cleft lip; prone in cleft palate

Esophageal Atresia with
Tracheoesophageal Fistula
 Esophgeal atresia is a failure of the esophagus to
form a continuous passage from the pharynx to
the stomach during embryonic development,
Tracheo-esophageal fistula is the abnormal sinous
connection b/w the trachea and esophagus.
 maternal hydramnios and prematurity
 S/S:
 coughing, choking, cyanosis, dyspnea, excessive secretion,
abdominal distention

 Dx:
 Ba swallow
Nursing Dx:
 Risk for imbalanced nutrition, less than body
requirements R/T inability for oral intake

 Risk for infection R/T aspiration or seepage of

stomach contents into lungs

 Risk for impaired skin integrity R/T gastrostomy

tube insertion site

Nursing care
 Suction regularly
 Elevate the head
 Gastrostomy feeding
 hydration
 O2

1. Observe for respiratory distress
2. Proper positioning –avoid hyperextension of neck
3. Continue suction
4. Prevent wound infection
5. Provide pacifier
Post Gastrostomy

Hirschprung’s Disease
 Aganglionic megacolon.
 Absence of parasympathetic
ganglionic cells in a segment of
the colon (usually at the distal
end of the large intestine:
rectosigmoid colon)
 Lack of innervation to a bowel
segment causes a lack of, or
alteration in, peristalsis in the
affected part
 Male predominance

 Causes
Familial, congenital defect

Commonly exists with other congenital

anomalies, particularly Down’s syndrome
(trisomy 21) and anomalies of the urinary tract
 Pathophysiology
 Absence of or reduce number of ganglion cells
in the intetinal muscle wall, distal end of colon.
 No paristalsis in the affected portion.
 Affected portion is narrow > proximal end
 Abddominal ddistention & constpation results. 23
 Constipation
 Ribbonlike stools
 Failure to pass meconium or stool
 abdominal distention
 Bile stained or fecal vomiting

 Complications
Severe Constipation
Enterocolitis – severe diarrhea, Hypovolemic shock, Death

 Dx:
Rectal biopsy provides definitive diagnosis by showing
the absence of ganglion cells
Ba enema – shows distention of the colon; spasm and a
narrowed lumen in the affected bowel
Abdominal X-rays show distention of the colon and
air-fluid levels; No air is seen in the rectum
 Tx: 1: primary resection of aganglionic segment.
2: colostomy, surgery: To decompress the colon, a
temporary colostomy or ileostomy may be necessary

Nursing Dx:
 Constipation R/T reduced bowel function
 Imbalanced nutrition, less than body requirements R/T reduced bowel

Nursing Care
1. Administer isotonic enemas: Normal saline solution or mineral oil to
evacuate the bowel
Daily enemas with 0.9% NaCl
Don’t administer tap water.
* Tap/hypotonic water will cause cardiac congestion or cerebral edema
2. Minimal residue diet with vitamin supplementation
3. Position semi fowlers to relieve dyspnea from distended abdomen
4. pacifier

After colostomy or Ileostomy
1. Monitor fluid intake and output (ileostomy will likely
cause excessive electrolyte loss)
2. Keep the area around the stoma clean and dry; use
colostomy or ileostomy appliances to collect drainage
3. Monitor for return of bowel sounds to begin diet

1. Observe for abdominal distention
2. Small frequent feedings after NGT removal
3. Colostomy care
4. Assist parents to cope with children’s feeding problems

 Is the invagination or
telescoping of the portion of the
intestine into an ajucent, more
distal section of the intestine.4-
10 months
 S/S: Intense abdominal pain,
vomiting, blood in stool “currant
jelly”, abdominal distention
(sausage shaped mass)
 Dx: sonogram “coiled spring”
 Tx: Ba enema (reduction by
hydrostatic pressure), surgery

Nursing Dx:
 Pain R/T abnormal abdominal peristalsis

 Risk for deficient fluid volume R/T bowel


Nursing Care

1. Provide comfort measures - pacifier for infants.

2. NPO.
3. Adequate hydration via IV therapy.

4. Insertion of N.G tube to deflate stomach.

5. Observe patient regularly for any complication.

6. Promote parent-infant bonding.
7. Post op nursing care.

Pyloric Stenosis
 Hypertophy/hyperplasia
of pyloric sphincter
 Males
 S/S: nonbillous vomiting,
s/s of dehydration and wt
loss, abdominal
distention, “olive” sized
mass RUQ, visible
 Dx: xray-”string sign”,
USG, endoscopy
 Tx: surgery
Nursing Dx:
 Risk for deficient fluid volume R/T inability to
retain food

 Risk for infection at site of surgical incision R/T

danger of contamination from feces R/T proximity
of incision to diaper area

Nursing care
 Hydration
 Pacifier
 may give thickened feedings on upright position then NPO
just before surgery
 Monitor I and O, weight, and vomiting

1. Dropper feeding 4-6 hrs after surgery 45 min- 1 hr
duration; oral rehydration soln then half strength
breastmilk/formula at 24 hr interval
2. Side lying position
3. Monitor weight and return of peristalsis
4. Wound care
5. Pacifier for oral needs
Hiatal or Diaphragmatic
 Part of stomach protrudes through the esophageal hiatus
of the diaphragm into thoracic cavity. OR
 Herniation of intestinal content into the thoracic cavity.

 Predisposing factors include:

• Congenital weakness
• Increased intra-abdominal pressure
• Increased age
• Trauma
• Forced recumbent position
Diaphragmatic Hernia

 Left side
 S/S: respiratory difficulty,
cyanosis, retractions, (-)
breath sounds affected
side, scaphoid abdomen
 Dx: CXR, Upper
 Tx: Diaphragmatic
Hernia repair

Nursing Dx:

Risk for ineffective airway clearance R/T displaced


Risk for imbalanced nutrition, less than body

requirements, R/T NPO status

Nursing Care
 Mechanical ventilation
 Elevate head
 Low intermittent suction

1. NICU care.
2. Semi-fowlers
3. Wound care
4. Maintain warm, humidified environment– lung fluid drainage
5. Suction prn
6. Chest physio
7. NPO – prevent pressure on diaphragm 39
Celiac Disease/Gluten sensitive
enteropathy/Celiac sprue

 Celiac disease also called gluten-induced enteropathy, is an

inborn error of metabolism Characterized by poor food
absorption and sensitivity or intolerance to gluten (a protein
found in grains : wheat, rye, oats, barley)

 Usually becomes apparent between ages 6-18 months

 Causes:
Gluten intolerance
Immunoglobulin deficiency

 A decrease in the amount and activity of enzymes in the
intestinal mucosal cells causes the villi of the proximal
small intestine to atrophy and decreases intestinal
 Malabsorption of fat-soluble vitamins (????), minirals &
some protiens & carbohydrates.

 Lymphoma of the small intestine
 Impair growth, succeptibility

 S/sx:
 steatorrhea (????) because of inability to absorb ____?
 chronic diarrhea
 anorexia
 malnutrition; weight loss
 failure to thrive
 coagulation difficulty from the malabsorption of fat soluble
 irritability
 anemic (Fe deficiency)
 abdominal pain and distention

 CELIAC CRISIS: constitutes acute medical
emergency and threat to life
 acute vomiting and diarrhea,dehydration &

 Dx:clinical; IgA antigliadin Ab ↑↑, gluten free diet

 Mx: gluten free diet, vitamin & Fe


 NDx: Imbalance nutrition: Less than body

requirements R/T impaired absorption
Nursing Intervention:
 nutrition counseling
 Eliminate gluten from the diet:
 avoid cereals, bread, cake, cookies, spaghetti, pizza,
instant soup, some chocolates

 Give the child: corn and rice products, soy and potato flour,
breast milk or soy- based formula, and fresh fruits.

 Replace vitamins and calories; give small, frequent meals.

 Monitor for steatorrhea- its disappearance is a good

indicator that the child’s ability to absorb nutrients is

Imperforate Anus

 Is the congenital presence of an intact anal

membrane or internal blind pouch of the bowel.

 An arrest in embryonic development of the anus,
lower rectum and urogenital tract at the 8th week
of embryonic life.
 Fistula is more likely to be formed.
 Others anomalies likely, especially TEF.
 Cause unknown.
Types ano-rectal malformations
 Anal stenosis.
 Imperforate anal membrane.
 Anal agenesis.
 Rectal agenesis.

 No anal opening.
 Absence of meconium stool.
 Green-tinged urine---if fistula is present.
 Progressive abdominal distention.

Treatment for
 Low-type imperforate anus.
• Local dilatation of fistula.
• Definitive repair.
• Anoplasty.

 High-type imperforate anus.

• Colostomy for decompression.
• Definitive pull-through surgery, deferred untill
1 year of age.
 Complications: before & after surgery
 Physical examination of newborn.
 No anal opening.
 Presence of perinial fistula.
 Presence of meconium-stained urine.

Pre-op nursing care:

 With-hold feeding, note any vomiting (COCA).
 N.G intubation.
 Measure abdoominal girth.
 Keep the baby warm.
 Keep fistula area clean.
 Observe for any compliction. 49
Post-op nursing diagnosis

 Risk for infection R/T surgical incision of anoplasty

 Risk for impaired skin integrity R/T ostomy

 Risk for fluid volume deficit R/T restricted intake

Poost-op nursing care
Depending on the location, type of imperforate anus,
sex of child.
 Prevent infection of suture line.

 Expose perinium to air.

 Proper positioning, prone or side to side.

 Observe for redness, drainage, poor healing.

 Preventing skin breakdown, (ostomy care).

 Maintaining fluid & electrolyte balance.

 Start oral feed as ordered (usually within hours

after of anoplasty).
 Moniter for return of paristalsis.

 Parentral fluids untill oral feed not started.

 Observe for abdominal distetion, bleedding from

perinium 51

 Sandra Nettina: The Lippincott Manual of Nursing Practice, 6th

edition, 1996 Lipponcott –Raven Publishers



Thank you!