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ARBAMINCH UNIVERSITY
COLLEGE OF MEDICINE AND HEALTH SCIENCE
SCHOOL OF NURSING
DEPARTMENT NEONATAL NURSING
INDIVIDUAL ASSIGNMENT ON NN TWO
BY:-AMBAYE BERHANU
ID.NO. PRHMS/011/2015
SUBMITTED TO: MR.AGEGNEHU B 1
SUBMISSION DATE: - DECEMBER 16, 2023
 Out lines
Introduction
Definition
Epidemiology

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Pathophysiology
Etiology
Clinical presentation
Diagnosis and differential diagnosis.
Management and nursing intervention 2

Summery and Reference

 Learning objectives
At the end of this unit, students will be able to:

Discuss the two most common abdominal wall defects and

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hirchispring diseases.

Identify the risk factors/causes of gastroschise,

omphalocele and hirchispring diseases.

Explain the pathophysiology of gastroschise, omphalocele

and hirchispring disease.

3
 Cont..

 Differentiate the characteristics b/n Gastroschisis and

Omphalocele.

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 Discuss about clinical manifestation of
omphalocele,gastroschise and hirschisprung disease.
 Provide appropriate nursing intervention for
gastroschis,omphalocele and hirshusprung disease.
 Manage neonates with gastroschis,omphalocele and
4
hirchispring disease.
 Introduction

Abdominal wall defects are a type of birth defect that

allow the digestive organs such as the stomach or

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intestines to protrude through an abnormal opening in
the abdomen.
Omphalocele and gastroschisis are the two main
types of abdominal wall defects and the result of
errors during embryologic development of the fetal
5
abdominal wall.
 Cont….

The represent unique disorders that have different

clinical manifestation

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Hirschsprung's disease (also called congenital a
ganglionic mega colon) occurs when some of your
baby’s intestinal nerve cells don’t develop properly,
delaying the progression of stool through the
intestines.
6
 History of Gasroschisi and omphalocele

1733 –James Calder(scottish neonatal surgeon) first

described Gastroschisis.
Gastroschise is derived from Greek word

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

“Gaster”(Gastro)meaning belly and “schisis”

meaning to tear or split from”
In 1634-Ambroise Pare(French barber surgeon)first

described Omphalocele.
Omphalocele derived from Latin word
7

“Omphalos” meaning prominence or navel.

 Gastroschisis

Definition
Gastroschise is a herniation of abdominal contents

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through abdominal defect located to right of umbilicus.
Gastroschisis is a congenital abdominal wall defect
characterized by the complete lack of closure of the
abdominal musculature.

8
 Cont…

 There is no transparent sac to protect the intestine in

gastroschisis.
 It may be associated with other gastrointestinal

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abnormalities such as mal rotation, atresia or
stenosis.
 Not commonly associated with other organ
anomalies.
 Infants have a high proportion of intrauterine 9

growth restriction.
 Epidemiology of gastroschise

The prevalence of gastrooschis is approximately 3 to

4 per 10,000 live births.

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The incidence of gastroschisis between male infants
is similar to that of females infants.
The incidence of gastroschise is higher in singleton
than in twin gestations, and higher in offspring of
non-Hispanic White mothers than non-Hispanic Black
10
mothers.
Studies of worldwide have consistently reported that
under 20 years age women have higher rate of
offspring with gastroschisis than the general obstetric
population.

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This is likely related to lifestyle factors that are more
common in young people (e.g., cigarette smoking, use
of recreational drugs, alcohol consumption, low body
mass index, increased frequency of genitourinary
infection). 11
 Cont….

In 10% of cases, gastroschisis is associated with

anomalies outside the GI tract.

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Higher risk of preterm delivery in pregnancies with
gastroschisis (28%) compared with those without
gastroschisis (6%).

12
 Cont.…

It does not appear to be inherited. Having one baby

with gastroschisis does not make it more likely that

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you would have another baby with the condition
Environmental exposure like tobacco use,cigerete

smocking and alcohol drinking.

Low socio economic status.

Low maternal age (age of mother below 20 years).

13
 Pathophysiology of gastroschise

During the fourth week of

human embryonic development, the lateral body wall

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folds of the embryo meet at the midline and fuse
together to form the anterior body wall.
However, in gastroschisis and other anterior body
wall defects, this fails to occur by either one or both
of the lateral body wall folds not moving properly to
meet with the other and fusing together. 14
This incomplete fusion results in a defect that allows
abdominal organs to protrude through the abdominal
wall, and the intestines typically herniate through the

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rectus abdominis muscle, lying to the right of the
umbilicus.
Several hypotheses have been proposed to explain the
pathogenesis of gastroschisis; all involve defective
formation or disruption of the body wall in the
embryonic period, with subsequent herniation of 15

bowel .
 Cont.…
Major hypotheses include:

Failure of mesoderm to form in the body wall

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Rupture of the amnion around the umbilical ring
and with subsequent herniation of bowel.
Abnormal involution of the right umbilical vein
leading to weakening of the body wall
Disruption of the right vitelline(yolk sac) artery
with subsequent body wall damage and gut 16

herniation.
 Ethology of gastroschise

The cause of gastroschisis is less clear

Gastroschisis is caused by a failure of the

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formation and development of the ventral body wall
during embryogenesis, resulting in herniation of the
bowel.
Rupture of Omphalocele.

Folic acid deficiency.

17
Drug exposures, for
example,aspirin,acetominophen and ibuprofen.
 Risk Factors of gastroschise

Maternal smoking (possibly due to placental

insufficiency and abnormal development of the

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vascular system)
Maternal age <20 years old

Environmental exposures e.g. Nitrosamines

In take of aspirin and ibuprofen during pregnancy

Alcohol consumption. 18

Socio-economic status.
 Clinical Features of gastoschise
Defect to the right of intact umbilical cord allowing

extrusion of abdominal content

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No covering sac

Bowels often thickened ,matted, and edematous

Evisceration of the bowel leads to mal-rotation

Constriction of the base may cause intestinal stenosis,

atresia, and volvulus.

Undescended testicles
19
 Complication of gastroschise

Reduced bowel motility and absorption

 Related vascular compromise caused by the

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herniated bowel.
Intestinal stenosis, short bowel, intestinal
atresia,perforation,necrosis or volvulus.
Necrotizing Enter colitis

Electrolyte imbalance
20
Clinical features of sepsis and anemia
 Diagnosis of gastroschise
Gastroschisis can be diagnosed during pregnancy or
after the baby is born.
During pregnancy, there are screening test (prenatal

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tests) to check for birth defects and other conditions.
Gastroschise can be detected by the end of the first
trimester (11 to 14 weeks) and certainly in the second
trimester.
 Prenatal detection rates of gastro chis are over 90% 21

as a result of routine second-trimester.

 Cont..

Ultrasound

 Maternal serum alpha-fetoprotein

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(MSAFP) assessment performed in Neural
tube defect, Abdominal wall defects,
duodenal or esophageal atresia.
Magnetic resonance imaging(MRI)
22
 Differential diagnosis of gastroschise

Omphalocele.

Ectopic cords.

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Limb-body wall complex.

 Cloacal exstrophy.

Umbilical hernia.

Bladder exstrophy.
23
Pentalogy of Cantrell.
 Management of gastroschise

Pregnancy Management

Once gastroschisis is diagnosed, fetal growth and

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amniotic fluid volume are charted via ultrasound at
3 to 4-week intervals starting at 24 weeks
gestation.
Oligohydramnios may be related to fetal growth

restriction and is a risk for cord compression,

while polyhydramnios may be predictive of bowel 24

atresia.
After delivery management

The perfusion of the herniated contents should be

carefully evaluated. If bowel ischemia or

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infraction suspected immediate surgical
consultation is indicated.

If the viscera are well perfused it is important to

next place a clear plastic bag over the exposed

bowel as a temporary covering to minimize
25
evaporative heat and fluid loss.
 Cont..

An orgiastic tube should be inserted to decompress the

stomach.

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Placement of peripheral intravenous access to provide
antibiotics and maintenance fluid is performed.
Intravenous fluids are administered and started in 80-
120ml/kg due to the exposed bowel fluid losses.

The airway should be evaluated and maintained.

26
 Cont.…
Pre operative Management

Heat management

Sterile wrap or sterile bowel bag

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 Radiant warmer

Fluid management

 IV bolus 20ml/kg LR/NS.

Nutrition

TPN(Central venous line)

27
Keep NPO
 Cont.…
Abdominal distention

 OG/NG TUBE

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Urinary catheter

Infection control.

Broad spectrum antibiotics.

Closure of the defect.

ABC management.
28
 Cont.…

Surgical Management of Gastroschisis

The primary goal of gastro chis is repair to

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return the exposed bowel and new organs to the
abdominal cavity while minimizing intestinal
injury or increased intra-abdominal pressure.
Two treatment options are present for
gastroschisis. The first is primary repair, and the
second is delayed closure 29
 Cont.…

Primary repair is a procedure of choice if the

abdominal contents will fit into the abdominal cavity.

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30
 Cont.…
Staged closure,(repair)

In 1969 Allen and wrenn adapted schusters

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technique to treat gastroschisis
A synthetic material is used to create a sac to cover

the abdominal contents.

31
Cont.…

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32
 Poste operative care of gastroschise

Monitoring of vital signs, cardiovascular and

respiratory status.

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Fluid and electrolyte balance.
Pain management.
Wound care
Monitoring bowel function.
Administer antibiotics.
33
Gastric decompression.
 Nursing intervention of gastrioschise
Monitor gastroschisis defect and oxygen saturation
of new born.

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Monitor neonate's central and peripheral perfusion
every 15 minutes prior to surgery.
Ensure Nasogastric Tube (NGT) is placed on free
drainage and aspirated hourly.
Document accurate fluid balance and undertake
34

regular assessment of hydration status.

Treatment algorithm of gastroschise

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35
 Omphalocele

Definition
Omphalocele is herniation of umbilicus through

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which abdominal contents protrude.
 Omphalocele is an opening in the center of the
abdominal wall where the umbilical cord meets the
abdomen

36
.
Omphalocele is a midline abdominal wall defect at
the level of the umbilical cord, from which abdominal
content herniates.

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The defect is covered by a three-layer sac of
amion, Wharton's jelly and peritoneum.
Omphalocele is classified into three categories: small,
giant and ruptured . The term “giant omphalocele”
(GO) is defined as an abdominal wall defect that
37
measures ≥5 cm or that contains ≥75% of the liver.
 Epidemiology of omphalocele

Omphalocele and gastroschisis are the most common

fetal abdominal wall defects in the world, with

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prevalence of approximately 2 and 4 per 10,000 live
births, respectively consistent with the worldwide
omphalocele prevalence of 2.6 per 10,000 births.
The prevalence of gastroschisis appears to be
increasing at a faster rate than that of omphalocele.
38
 Cont.…

The prevalence is slightly higher in Black than White

patients.

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Modest associations between occurrence of
omphalocele in offspring and maternal obesity and in
utero selective serotonin reuptake inhibitor (SSRI)
exposure have also been reported in some studies,
while others have found no association.
39
 Cont..

Omphalocele has also been associated with male sex

and multiple births.

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The occurrence of omphalocele appears to be more
common in offspring of mothers at the extremes of
reproductive age
Omphalocele associated with congenital anomalies
such as bowel atresia, Imperforated anus, Trisomy's
40
13,18,21,Neural tube defect and cleft lip/palate.
Pathophysiology of omphalocele

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41
Ethology of omphalocele

No know exact causes of omphalocele, but its

development has been linked with several genetic

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and chromosomal abnormalities.
It might also be influenced by other factors, such
as the mother’s habits during pregnancy, or it could
happen due to a combination of reasons

42
Riske factors of omphalocele
These factors can increase the risk of an omphalocele in

the fetus:

Alcohol use. Alcohol consumption during pregnancy

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is linked to omphalocele.

Tobacco use. Women who use tobacco while

pregnant are at an increased risk of their unborn child

developing omphalocele.

Increased maternal age more than 40 years 43


Consecutive birth

Selective serotonin-reuptake inhibitors

(SSRIs) use during pregnancy are more

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likely to have a baby with omphalocele.
SSRIs are usually found in medications like
antidepressants.
Twins and
44
Obesity.
 Clinical Manifestation of Omphalocele
Central abdominal wall defect

Extrusion of intra-abdominal organs through the

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umbilicus
Herniated organs are usually small bowel and may
include large bowel and/or liver.
The cord inserts into the apex of the sac

Ascites may be seen in the sac or the abdomen. 45

 Cont…
Organs are covered by peritoneum
The umbilical cord insert directly in to the sac in an
apical or lateral position

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46
 Diagnosis of omphalocele
Prior to delivery
Perinatal ultrasound is diagnostic in 95% of cases

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Prenatal ultra sound after 14weeks gestation is the
confirmatory test

Increased maternal AFP( alpha-fetoprotein),This may

be as a result of direct protein loss from the intestine

into the surrounding amniotic fluid .

47
After delivery : Clinically
 Differential diagnosis of omphalocele
Gastroschisis: gastroschisis is the major disorder to
consider in differential diagnosis of omphalocele.

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Umbilical cord hernia.

Ectopia cords.

 Limb-body wall complex.

Cloacal exstrophy and

Pentalogy of Cantrell. 48
 Complication of omphalocele

Sepsis
Gastroesophagal reflux disease

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Necrotizing entercolitis
Intestinal atresia
Short gut syndrome
Renal vein thrombosis
Respiratory failure
Renal failure and Complication of total parenteral 49
nitration
Management of omphalocele

Pregnancy management

 Fetal surveillance

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In fetal surveillance monitoring fetal growth and

amniotic fluid volume.

When growth is appropriate and amniotic fluid

volume is normal, we begin weekly no stress

testing or biophysical profile monitoring at 32
50
weeks of gestation to assess fetal well-being.
 Cont.…
We increase the frequency of fetal surveillance in

pregnancies with additional complications, such as

growth restriction, oligohydramnios, or non isolated

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omphalocele.
Timing of birth

In the absence of standard indications for early

delivery, expectant management is reasonable until

spontaneous labor or at least 39 weeks of gestation. 51
 Cont.…
Preterm birth offers no advantage to affected

neonates and is associated with increased morbidity

and mortality.

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Route of birth

We perform a cesarean birth in selected cases,

including fetuses with giant omphalocele

52
 Cont.…
After Delivery

The initial evaluation &resuscitation to a babies

with an Omphalocele is

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Follow same protocol &sequence of all newborns

 Should be handled carefully to prevent the

Omphalocele membrane from tearing

Avoid clamping the umbilical sac.

53
 Cont.…
After initial stabilization for the new born
should be inspected to confirm that it is intact &
then covered with anon adherent dressing to

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protect the sac.
Covering the defect with a sterile dressing
soaked with warm saline to prevent fluid loss.
NGT decompression
54
Cont.…

IV fluids and glucose

Antibiotics

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Keep NPO until the surgical opinion obtained

Urgent surgical consultation

Surgical management

55
 Cont.…

Primary closure
In infants with small defects,.

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Small defects (2 to 3 cm) can be repaired in the first

24 to 72 hours of life by primary closure of both fascia

and skin.
Some surgeons prefer to place a patch in the

abdominal wall and close the skin over the patch

56
 Cont.…

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Figure ,Surgical management of Primary closure
of omphalocele
57
 Cont..

In infants with large defects

Involves some type of silo in the first 24 hours of

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life and delayed closure.
Reducing a large defect or one that contains an
anatomically kinked liver can be done with Doppler
ultrasound guidance to ensure the vena cava and
hepatic outflow are not compromised during the silo
58
reduction.
 Cont.…
Omphaloceles (i.e., containing >75 percent of the
liver or defect greater than 5 cm) may be managed
by a combination of silo, a cellular dermal patch, and

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skin graft or by promoting formation of an amniotic
sac escher by application of a sclerosing solution
(topical povidone-iodine), with delayed hernia
repair.

59
 Poste operative care of omphalocele

Monitoring of vital signs, cardiovascular and

respiratory status.

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Fluid and electrolyte balance.

Pain management.

Wound care

Monitoring bowel function and urine out put.

Administer antibiotics. 60

Gastric decompression.
Differentiating characteristics between
Gastroschisis and Omphalocele

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61
Cont.…

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62
 Hirsch sprung disease(congenital a ganglionic
mega colon)
Definition
Hirschsprung disease (HD) is a motor disorder of the

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colon, which is caused by the failure of neural crest cells
(precursors of enteric ganglion cells) to migrate
completely during intestinal development during fetal
life.
It is the result of a ganglionic segment of the colon
63
fails to relax, causing a functional obstruction.
Hirschsprung's disease (also called congenital a

ganglionic mega colon) occurs when some of your

baby’s intestinal nerve cells (ganglion cells) don’t

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develop properly, delaying the progression of stool

through the intestines.

The intestine becomes blocked with stool, and your

baby or child will be constipated. Often, a serious

infection called entercolits can occur, which causes 64

fever, pain, and diarrhea.

 Cont.…

In a healthy bowel, ganglion cells are present

throughout the large intestine. In Hirschsprung’s

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disease, ganglion cells don’t develop properly in the
rectum, delaying the progression of stool.

65
Epidemiology of HD

HD occurs in approximately 1 in 5000 live births,

with an overall male: female ratio of 3:1 to 4:1 .

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The sex ratio decreases with more extensive
aganglionosis, approaching 1:1 for total colon
aganglionosis.
There is familial clustering for nonsyndromic HD,
with an overall recurrence risk of approximately 3
percent in siblings for short-segment disease or up to
17 percent if the proband has long-segment disease.
66
Pathophysiology of HD

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67
Ethology OF HD

There is no known clear idea why the ganglion

cells don’t migrate down to the end of the rectum

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completely. However, genetic factors may be
involved, especially when longer lengths of
intestine are involved or when someone else in
the family also has the condition.

68
Cont.…

For instance, there is an increased chance that a

couple will have a child with Hirschsprung's

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disease if one of the parents has the disease. (The
chances are higher if the mother is the one with
Hirschsprung's disease).
If a family has a child with Hirschsprung's
disease, there is a 3 to 12 percent chance that
another baby from the same parents will also
have the disease.
69
Cont.…
Hirschsprung's disease occurs five times more
frequently in boys than in girls.

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Children with Down syndrome have a higher risk as
well.

70
Clinical manifestation of HD

In neonate and infants

Failure to pass meconium in 48 hours.

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Abdominal distention
Bile stained vomiting.
Episodes of diarrhea and constipation
Poor feeding.
 Tight anal sphincter with an empty rectum
Shock and jaundice
71
 Cont..

Older infants or children

Constipation with abdominal distention

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When stool passed foul smelling and liquid
in consistency.
Malnourished and anemic.
Poor feeding.
Fecal impaction and
Failure to thrive.
72
Cont..

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73
Diagnosis of HD
Hirschsprung disease can be diagnosed by taking a
medical and family history, doing a physical exam,
and ordering tests. Although Hirschsprung disease

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develops before birth, doctors cannot diagnose the
disease until after an infant is born.
About half the people with Hirschsprung disease are
diagnosed in their first year of life.
About 80% of people with Hirschsprung disease are
diagnosed by age 7, and more than 90% are
diagnosed by age 13.
74
Cont.…

Medical and family history.

Physical exam

01/09/24
During a physical exam, a doctor typically
Reviews your child’s height and weight
Examines your child’s abdomen or belly for
swelling.
Performs a digital rectal exam having no stool
in the rectum or having explosive stool after a
rectal exam may be signs of Hirschsprung
75
disease
Cont.…

01/09/24
During a physical exam, a doctor typically
examines your child’s abdomen for swelling 76
Cont..

To diagnose Hirschsprung disease use a

combination of :

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Imaging tests
Anorectal manometer and
Rectal biopsies.
Imaging tests
 To check for signs of Hirschsprung disease,
doctors may use imaging tests such as x-ray
and ultrasound to view large intestine. 77
Cont.…
Anorectal manometer
 Anorectal manometer is a test that checks how

01/09/24
well your child’s rectum is working.
During the procedure, the doctor inflates a
small balloon inside your child’s rectum.
Normally, the muscles in the rectum will relax.
 If the muscles don’t relax, the doctor may
suspect your child has Hirschsprung disease.

78
Cont.…
Rectal biopsy
Doctors typically use rectal biopsies to confirm

01/09/24
or rule out a diagnosis of Hirschsprung disease.
During a rectal biopsy procedure, a doctor will
take small pieces of tissue from the rectum.
A pathologist will examine the tissue under a
microscope to look for signs of Hirschsprung
disease.
79
 Differential diagnosis of HD

Meconium ileus.
Anorectal stenosis and

01/09/24
Pelvic tumors.
Anorectal malformation.
 Neonatal sepsis.
Intestinal atresia,
Anorectal anomalies,
Duodenal atresia and pyloric stenosis.
80
 Management of HD

Medical management
Intravenous hydration,

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 Intestinal and gastric decompression.
Administration of isotonic enema.
Administration of stool softeners
Low residue diet
Antibiotics and
Rectal irrigation.
81
 Cont.…

Pre operative management of HD

Assess complete history of new born

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After the diagnosis counseling the family
Nurse thought about giving isotonic enema,
suppositories and stool softeners.
 Monitor vital sign and abdominal girth of child.
Keep the child in semi fowlers position.
Keep NPO and insert NG tube.

82
 Cont.…

Surgical management .
Surgery to bypass or remove the affected part of

01/09/24
the colon is necessary for the patient to have proper
bowel movements. Several options exist for
surgical treatment of HD.
A pull-through surgery removes the diseased part
of the colon or bowel.
The section with nerve cells is then pulled through
the colon and replaces the lost area.
83
 Cont.…

01/09/24
Pull-through surgery is the primary treatment for Hirschsprung disease

disease to remove the affected area of the bowel 84

Ostomy(colostomy) surgery is an additional first step
in very sick patients who cannot yet have the pull-
through procedure.

01/09/24
The ostomy method removes the abnormal portion of
the colon while the healthy colon is routed to a
surgical opening or “stoma” on the abdomen
Ostomy pouches are then attached to this abdominal
port from which the stool is passed, collected and
emptied. 85
 Cont.…

Once the colon has had time to heal in this position,

the stoma is closed and the pull-through procedure is

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completed.
If severe damage has occurred due to inflammation,
the ostomy pouch system may be a permanent
solution to prevent further damage.

86
 Cont.…
An ostomy is performed to allow patients to pass
digestive waste as the bowel heals from Hirschsprung
disease

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87
Cont.…
Poste operative management
Monitor vital signs, observe abdominal bleeding.

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Keep NPO, so administer Iv fluids as ordered
Monitor bowel sound
Colostomy care is to be done which include to
Observe for bleeding, purulent drainage, edema and
apply zinc oxide ointment on skin around stoma.

88
` Cont..

Frequently empty the collecting bag.

Keep colostomy clean and dry
Educate parents about colostomy care

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Encouraging and supporting the family during this
stressful time is the key intervention of nurse.

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Nursing Interventions of HD

Improving Breathing Pattern

Administer oxygen, as ordered, to support
respiratory status

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Relieving Pain
Providing Adequate Nutrition
Controlling Constipation in the Older Child
Preventing Complications Related to Colostomy

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 Summery

 Abdominal wall defects are type of congenital

defect that allows the stomach, intestines, or

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other organs to protrude through unusual
opening that forms on the abdomen.
 Omphalocele and gastrischisis are the
commonest congenital abdominal wall defects.
 The disease do have embryological origin is the
current most accepted theory for Omphalocele is
failure of the midgut to return back to the cavity
91
 Cont.….
Primary closure or staged closure can be used by
using different criteria's for both condition

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Hirschsprung’s disease is a congenital
malformation which is characterized by a lack of
ganglion cells in the intestine which prevents
peristaltic activity through the bowel.
Abdominal distension, bilious vomiting and/or
enterocolitis and an X-ray that suggests distal
bowel obstruction may suggest Hirschsprung’s
disease. 92
 Cont.…

Diagnosis is confirmed by rectal examination,

and suction rectal biopsy.

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The most commonly used surgical procedure to
treat Hirschsprung’s Disease at the Children’s
Hospital at West mead is the Soave procedure
where normal intestine is “pulled through” to the
anus
Long segment or complex disease may be
initially managed with a stoma.
93
 Cont.…
Once the colon has had time to heal in this position,
the stoma is closed and the pull-through procedure is
completed.

01/09/24
If severe damage has occurred due to inflammation,
the ostomy pouch system may be a permanent
solution to prevent further damage.

94
 References

 Nelson Textbook of Pediatrics 21 edition

 Neonatal Clinical Practice Guidelines .

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 Up to date.

 Medscape.

 Aschcraffs pediatric surgery 6th edition.

 Lippincott manual of nursing practice 10th edition.

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