Uploaded by Mohamed Na3eem

√ Congenital Anomalies of Gastrointestinal Tract

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Dr
/
Hanaa Mabrouk Ahmed


Identify the definition of common diseases of
the gastro-intestinal tract.
List predisposing factors, manifestations and
treatment of gastro-intestinal disorders.




Differentiate between clinical signs &
symptoms for common diseases of the
gastro-intestinal tract .
Evaluate different child condition in
diseases of the gastro-intestinal tract
Analyze any complication, and implement
proper intervention.
Distinguish between different diseases of the
gastro-intestinal tract.




Appreciate parent roles in management of
gastrointestinal diseases
Carry out nursing care plan for different
diseases of the gastro-intestinal tract
Be responsible about the management of
gastrointestinal diseases with cooperation of
medical team.
Design nursing care plans and case studies
for different diagnosis

Participate in giving and counseling about
emergency care

Reassure caregivers about condition of their
children
1-Lecture
2-Group-Discussion
3-Class participation
4-Brain storming
5-Audiovisual with computer assisted
6-Case Study
1-Introduction
2-Causes
3- Functions of the Gastrointestinal System
4-The most common anomalies
a- Congenital Hypertrophic Pyloric Stenosis
b- Hirschsprung’s (congenital aganglionicmegacolon).
c- Intussusception.
5- Follow up activities
6- References

Birth defects are also called "congenital
anomalies" or "congenital abnormalities." The
word "congenital" means "present at birth."
The words "anomalies" and "abnormalities"
mean that there is a problem present in an
infant.

Unknown

Inheritance

Chromosome Abnormalities:

A congenital metabolic disease

Teratogens: A teratogen is an agent, that can cause a birth
defect. It is usually something in the environment that the
mother is exposed to during her pregnancy. It could be a
prescribed medication, a street drug, alcohol use or a
disease that the mother has, which can increase the chance
for the baby to be born with a birth defect.

Hypertrophic Pyloric stenosis (HPS)

Hirschsprung's disease

Intussusception

Pyloric stenosis is an unusually narrow opening (stenosis)
between your child’s stomach and small intestine (pylorus).
Normally, food moves easily from the stomach into the small
intestine through the pylorus. If the muscles of the pylorus are
thicker than normal (hypertrophy), this makes the opening
narrow and prevents food from passing easily out of the
stomach. Pyloric stenosis can cause almost complete blockage
of this opening. Pyloric stenosis usually develops in the first
few weeks after birth. The most common sign is very forceful
(projectile) vomiting soon after a feeding.

It is a congenital hypertrophy of circular muscle
layer of pyloric canal leading to stenosis of its
lumen.

The symptoms of this condition usually start at
the age of 2-6 weeks of life resulting in partial or
complete obstruction of the stomach outlet.

The exact cause is unknown.

Incidence

2: 1000 live births

Male: female 4:1 (particularly first-born males).

Risk

The risk of pyloric stenosis may be greater if:

Age: is between 3–6 weeks old.

Gender: is male.

There is a family history of pyloric stenosis.

The mother uses tobacco during pregnancy.

The circular muscle of the Pylorus as a result of the
hypertrophy produces severe narrowing of the pyloric
canal between the stomach and the duodenum.

-Hypertrophy of circular muscle layer of pyloric canal.

-Narrowing of pyloric canal.

Elongated pyloric canal.

Dilated stomach.

Multiple superficial gastric ulcerations.

The symptoms of pyloric stenosis are most frequently observed between the age of
3 and 6 weeks, but may well appear earlier.

Vomiting Is Often Described As Non-Bile Stained ("Non Bilious") And "Projectile
Vomiting",

Poor Feeding

Weight Loss

Dehydration

Constant Hunger, Belching, and colic

Visible gastric peristalsis in the upper left abdominal quadrant from left to right (from
the left subcostal margin to the umbilicus).

Palpable pyloric mass (Olive like mass felt just beneath the outer border of the right
rectus muscle at the level of the umbilicus).
Initial treatment

Rehydrate and correct electrolytes

Correct alkalosis

Surgical pyloromyotomy (Ramstedt
procedure)
Preoperative Care

Nothing per mouth, and naso-gastric intubation for
suction and repeated washing using warm saline

Intravenous fluid therapy to correct dehydration and
electrolyte disturbances.

Intravenous broad-spectrum antibiotics and vitamin
K.
Operation:
 Pyloromyotomy
operation)
(Ramestedt’s

Measuring vital signs

Observe for postoperative complication

Monitor parenteral fluids to maintain hydration

Oral feeding resumed 2-8 hours after surgery (slowly)

if the mother was breast feeding, she should resume this as soon as possible.

Report any vomiting

Removal of the naso-gastric tube after 12 hours post-operatively.

Very gradual oral feeding starting by 5 ml of dextrose 5%, if tolerated the amount is
increased in the next feed. Gradual Shifting to ½ strength milk formula, if tolerated
well full strength milk formula is given gradually also 36-48 hours, and then the baby
can be discharged with his mother with breast feeding.
Pyloric stenosis can lead to:

Failure to grow and develop.

Dehydration. Frequent vomiting can cause dehydration and a mineral
(electrolyte) imbalance. Electrolytes help regulate many vital functions.

Stomach irritation. Repeated vomiting can irritate your baby's stomach and
may cause mild bleeding.

Jaundice. Rarely, a substance secreted by the liver (bilirubin) can build up,
causing a yellowish discoloration of the skin and eyes

It is a rare condition in which nerve cells that control
squeezing movements (contractions) are missing from
part of the large intestine (colon). The colon absorbs
fluids and holds stool so that it can be passed out of
the body with contractions.

In Hirschsprung disease, the colon has trouble
moving stool out through the rectum. This leads to
constipation.

Hirschsprung disease (HD) is a congenital
absence of the parasympathetic ganglion
nerve cells from within the muscle wall of
the intestinal tract usually at the distal end
of the colon resulting in a functional
obstruction.

Hirschsprung's disease occur in 1 every 5000 live
births

The basic pathological finding in Hirschsprung's
disease is the absence of ganglion cells
from the
distal part of the rectum and extending proximally for a
varying distance. This a ganglionic segment is nonpropulsive and fails to relax resulting in functional
intestinal obstruction. The proximal colon is grossly
dilated due retained stool, its wall is hypertrophied,
and its mucosa is edematous and inflamed (Entero-
colitis).

The cause of this condition is not known, but it may
be related to changes (mutations) in genes. It may
run in families or be passed from parent to child
(inherited) through genes.

In children who have the disease, it is present from
birth (congenital). It happens when nerve cells in
the colon stop growing while the colon is
developing before birth.

Hirschsprung's disease is usually diagnosed shortly after birth, but in some milder cases,
symptoms don't develop until later in life. Symptoms in older children include the following:

Constipation or gas, which may make your baby fussy

Poor appetite

Vomiting bile (a green liquid) after eating

Diarrhea

Chronic constipation

Lack of weight gain or growth

Swollen abdomen

Ribbon-like, foul-smelling stools

Anemia (which is a low number of red blood cells)

Frequent colon infections

Abdominal distension

Thin abdominal wall (superficial veins are visible)

Visible peristalsis

Constipation

Foul-smelling stools

Failure to growth

This condition is diagnosed based on:

child's symptoms.

A physical exam.

An X-ray of: child's colon after dye has been put into the colon
(barium enema).

Removal of a colon tissue sample to be examined under a microscope
(biopsy).

Measuring pressure in the rectum by inserting a balloon and inflating it
(anal manometry).
Goals

Manage acute bowel obstruction

Correct volume deficits and restore electrolyte balance

Decompress gastrointestinal tract

Recognize and eradicate any coexisting infections (eg,
enterocolitis)

Initial surgical goal is to eliminate functional
obstruction caused by aganglionic bowel

Ultimate surgical goal is to reconstruct the
intestine, connecting normally innervated
bowel to the anal opening

Surgery is required for definitive treatment

Antibiotics are given for any cases of
suspected enterocolitis

IV fluids

Total parenteral nutrition for nutrient
and calorie support if necessary

Gastrointestinal
decompression
intestinal obstruction is present
if
A. Preoperative care
If the operation is scheduled (elective),

a bath or shower is recommended the night before
or the morning prior to surgery.

The child will not eat anything for 6-8 hrs before
surgery.

Intravenous antibiotics will be given in the operating
room.
1- Skin Care

After the operation for Hirschsprung's disease,
children who do not have a stoma will have a lot of
diarrhea. It is important to protect the skin around
the anus at all times to prevent irritation, using a
cream called "butt balm." child's nurse will teach
you how to mix and apply the cream.

2-Dressings

If the child has an incision on the abdomen, there will be a
gauze pad and clear plastic dressing in place.

You should remove this dressing two days after the operation.

There may be a small amount of blood on pieces of tape, called
Steri-strips, under the dressing. This is normal.

You can remove the Steri-strips one week after the operation.
Child can bathe with the Steri-strips in place.
3-Medications
Most children don't need prescription pain medication after they're
discharged from the hospital. Acetaminophen (Tylenol) or ibuprofen (Motrin) is
usually sufficient. Administer the medication according to the dosage
directions on the label. If the child is still uncomfortable, call our office and we
may prescribe something stronger.
4-Activities
The child can return to his or her usual activities without any
special restrictions, unless the pediatric surgeon tells you
otherwise. School-age children can resume their school activities
as soon as they feel well enough

Diet: child may eat a normal diet after surgery.

Activity:
child should avoid strenuous activity and heavy
lifting for the first week after laparoscopic surgery, 4-6 weeks
after open surgery.

Wound care: Surgical incisions should be kept clean and dry
for a few days after surgery. Most of the time, the stitches used
in children are absorbable and do not require removal. Your
surgeon will give you specific guidance regarding wound care,
including when your child can shower or bathe.

.

Medicines: Medicines for pain such as acetaminophen or something
stronger like a narcotic may be needed to help with pain for a few days after
surgery. Stool softeners and laxatives are needed to help regular stooling
after surgery, especially if narcotics are still needed for pain.

What to call the doctor for: Call doctor for worsening belly pain,
fever, vomiting, diarrhea, problems with urination or if the wounds are red or
draining fluid.

Follow-up care: the child should follow up with his or her surgeon 2-3
weeks after surgery to ensure proper postoperative healing
Definition

Intussusception
is
the
invagination
or
telescoping of a portion of the intestine into an
adjacent, more distal section of the intestine.
Etiology

Not usually known

May be due to increased mobility of intestine and
hyper peristalsis

possible contributing factors include:
◦ Meckel's diverticulum
◦ Malrotation of intestine
◦ Acute gastroenteritis
◦ Abdominal injury
Incidence

Intussusception usually occurs in 6 months – 2
years infants.

Boys :girls is 2:1.
Pathophysiology

It occurs when a segment of small bowel
"telescopes" on itself, thus causing swelling,
obstruction,
gangrene.
and
eventually
intestinal

Intussusception preventing the passage of food that is
being digested through the intestine.

The walls of the two "telescoped" sections of intestine
press on each other, causing irritation and swelling.

Eventually, the blood supply to that area is cut off,
which can cause damage to the intestine.
Clinical manifestation
Early signs/symptoms:
(1) intermittent episodes of sudden onset of severe
abdominal pain that lasts only a few minutes. Infants
may pull up their legs during episodes of pain;
(2) vomiting;
(3) abdominal mass in the right upper side of the
abdomen; suzage shaped mass
(4) lethargy due to dehydration.
Later signs/symptoms:
(1) bloody stools with a mucus-like texture that
resemble currant jelly;
(2) bilious (green) vomiting from intestinal obstruction;
(3) fever, low blood pressure, and fast heart rate can be
signs of bowel perforation
Investigations
 Plain
X-ray
 Ultra
sound
 Barium
enema

Hydrostatic reduction of telescoped bowel with a
barium enema during the 1st 48 hours after
onset.

Surgical reduction of Intussusception, resection
if bowel is gangrenous.

Baseline observations include heart rate, respiratory rate, temperature and pain score. Blood
pressure, oxygen saturations and neurological observations if clinically indicated.

Minimum of 1 hourly observations

Any significant changes should be reported immediately to the medical team

Ensure appropriate medication, monitoring, oxygen and emergency equipment is available for
transfer and reduction procedure

Fluid input/output is to be monitored and documented.

Enema

Gastric suction

Antibiotics
Symptomatic

Fluids intravenously until bowel function returns;
he or she may then be allowed to resume a
normal diet.

Follow-up
care
may
be
indicated
if
the
intussusception occurred as a result of a specific
condition (e.g., cancerous tumors).

Perforation of bowel, with peritonitis

Necrosis of bowel requiring bowel resection

Shock and sepsis

Re-intussusception after spontaneous or active
reduction.
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