WESLEYAN UNIVERSITY-PHILIPPINES

AN AUTONOMOUS METHODIST UNIVERSITYCOLLEGE OF

NURSING

A CASE STUDY IN:

HIRSCHSPRUNG
DISEASE

Submitted by:
Jaimie D. La Peña
 WESLEYAN UNIVERSITY-PHILIPPINES
AN AUTONOMOUS METHODIST UNIVERSITYCOLLEGE OF
NURSING

TABLE OF CONTENTS

Title Page
Table of Contents
I. Case Scenario
II. Patient’s Profile and History
III. Anatomy and Physiology
IV. Overview of the Disease
a. Definition
b. Etiology
c. Risk Factors
d. Clinical Manifestations
e. Types of Hirschsprung's disease
f. Diagnostic Procedures
V. Pathophysiology
VI. Management
a. Medical Management
b. Surgical Management
c. Nursing Management
d. Discharge Planning
VII. Nursing Care Plan
VIII. Drug Studies
a. Bisacodyl
b. Ampicillin
References
 WESLEYAN UNIVERSITY-PHILIPPINES
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I. Case Scenario
A 9 years old, previously healthy boy presented with sudden onset of colicky abdominal
pain of 4 h duration. The pain was mainly infra-umbilical. Associated with this he had gross
abdominal distension and 2 episodes of non-bilious vomiting. Parents reported that the child had
bowel movements daily before he joined school since when bowel movement became every 2–
3 days. The child had similar attack a month before presentation to our hospital and rectal tube
deflation was done at a nearby hospital. Otherwise there was no other complaints. His growth
and development is similar compared to his peers. He was not on any medications. There was no
family history of similar illnesses.
On physical examination except for tachycardia other vital signs were all in normal
range. All anthropometric measurements were in normal range. He was in pain while holding his
abdomen. He had gross abdominal distension. There was no direct or rebound tenderness.
Abdomen was hyper tympanic to percussion. Bowel sounds were increased. There was no
organomegally or palpable masses on deep palpation. Per-rectum examination showed empty
rectum. No stool or blood on examining finger. A Plain abdominal radiographs showed single
hugely dilated bowel loops in the left lower quadrant with single air fluid level (Fig. 1).
Abdominal ultrasound was unremarkable except for gas filled abdomen. Complete blood count
and organ function test was done and all were in normal range.
We put the patient in knee-chest position and gently introduced fully lubricated rectal
tube into the rectum and slowed advanced upward. Gush of air and scanty stool came out. The
abdomen became soft and the cramp subsided. We left the tube in the rectum for 24 h. We took
full thickness rectal biopsy the next day. It showed aganglionated rectum with absent ganglion
cell in both submucosal and muscles layers. We also obtained diagnostic barium enema and it
showed redundant sigmoid with no radiologic evidence of Hirschsprung’s disease (Fig. 2).

Figure 1. PA plain abdominal x-ray showing Figure 2 . PA barium enema study showing dilated

single air fluid level with coffee bean ap and redundant sigmoid colon without radiologic evidence
of
Hirschsprung’s disease
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II. Patient’s Profile and History

A. BIOGRAPHICAL DATA
Patient’s Profile
Name: Patient X
Age: 9 years old
Sex: Male
Date of Birth: July 21, 2013
Address: Cabanatuan City
Civil Status: Single
Religion: Roman Catholic
Nationality: Filipino
Attending Physician: Dr. X
Chief Complaint: Abdominal pain of 4 h duration, abdominal distension, 2 episodes
of non-bilious vomiting, bowel movement became every 2–3 days
Admitting Diagnosis:
Final Diagnosis:

Patient’s History
Past Medical History: Had similar attack a month before presentation to our hospital and
rectal tube deflation was done
Family History: None
Allergies: No known allergies
Surgical: Not Stated
Medications: None
Vitals: Blood Pressure 120/80, Pulse Rate 112, Respiratory rate 18,
Temperature 37 degree Celsius
Injuries and Accident: Not Stated
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III. Anatomy and Physiology

The digestive system is made up of the
gastrointestinal tract—also called the GI tract
or digestive tract—and the liver, pancreas,
and gallbladder. The GI tract is a series of
hollow organs joined in a long, twisting tube
from the mouth to the anus. The hollow
organs that make up the GI tract are the
mouth, esophagus, stomach, small intestine,
large intestine, and anus. The liver, pancreas,
and gallbladder are the solid organs of the
digestive system.
As food moves through your GI tract,
your digestive organs break the food into
smaller parts using:
motion, such as chewing, squeezing, and
mixing
digestive juices, such as stomach acid, bile,
and enzymes
Mouth. The digestive process starts in your mouth when you chew. Your salivary glands make
saliva, a digestive juice, which moistens food so it moves more easily through your esophagus
into your stomach. Saliva also has an enzyme that begins to break down starches in your food.
Esophagus. After you swallow, peristalsis pushes the food down your esophagus into your
stomach.
Stomach. Glands in your stomach lining make stomach acid and enzymes that break down food.
Muscles of your stomach mix the food with these digestive juices.
Pancreas. Your pancreas makes a digestive juice that has enzymes that break down
carbohydrates, fats, and proteins. The pancreas delivers the digestive juice to the small intestine
through small tubes called ducts.
Liver. Your liver makes a digestive juice called bile that helps digest fats and some vitamins.
Bile ducts carry bile from your liver to your gallbladder for storage, or to the small intestine for
use.
Gallbladder. Your gallbladder stores bile between meals. When you eat, your gallbladder
squeezes bile through the bile ducts into your small intestine.
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Small intestine. Your small intestine makes digestive juice, which mixes with bile and
pancreatic juice to complete the breakdown of proteins, carbohydrates, and fats. Bacteria in your
small intestine make some of the enzymes you need to digest carbohydrates. Your small intestine
moves water from your bloodstream into your GI tract to help break down food. Your small
intestine also absorbs water with other nutrients.
Large intestine. In your large intestine, more water moves from your GI tract into your
bloodstream. Bacteria in your large intestine help break down remaining nutrients and make
vitamin K. Waste products of digestion, including parts of food that are still too large, become
stool.
IV. Overview of the Disease
a. Definition
Hirschsprung's disease is a condition that affects the large intestine (colon) and causes
problems with passing stool. The condition is present at birth (congenital) as a result of missing
nerve cells in the muscles of the baby's colon. Without these nerve cells stimulating gut muscles
to help move contents through the colon, the contents can back up and cause blockages in the
bowel.
b. Etiology
During fetal development, nerve cells typically grow from the top of the small intestine
through the large intestine to the anus. In children with Hirschsprung disease, these nerve cells
stop growing in the large intestine before reaching the anus. Doctors aren’t sure why this
happens.
About 20% of the time, Hirschsprung disease runs in families. A change in a gene (genetic
mutation) may be the cause. A parent with Hirschsprung has a higher risk of having a child with
the disease. And if one child in a family has the condition, siblings are more likely to have it, too.
c. Risk Factors
Factors that may increase the risk of Hirschsprung's disease include:
Having a sibling who has Hirschsprung's disease. Hirschsprung's disease can be
inherited. If you have one child who has the condition, future biological siblings could be at risk.
Being male. Hirschsprung's disease is more common in males.
Having other inherited conditions. Hirschsprung's disease is associated with certain
inherited conditions, such as Down syndrome and other abnormalities present at birth, such as
congenital heart disease.
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d. Clinical Manifestations

Children with Hirschsprung disease who don’t have signs and symptoms of intestinal
obstruction at birth may not be diagnosed and treated until they are older. Common symptoms in
older infants and children include

 chronic constipation that doesn’t get

better after treatment with
oral laxatives—laxatives that are
taken by mouth
 Abdominal pain and swelling
 Brown or green vomit
 malnutrition or failure to thrive,
meaning that an infant or child weighs
less or is gaining less weight than
expected for his or her age

In older infants and children, symptoms of Hirschsprung disease, such as constipation, may
be similar to symptoms of other conditions. Therefore, doctors may not suspect
and diagnose Hirschsprung disease right away.

In rare cases, Hirschsprung isn’t diagnosed and treated until adulthood. Adults with
Hirschsprung disease typically have a history of abdominal swelling and chronic constipation
that doesn’t get better after taking oral laxatives.

e. Types of Hirschsprung's disease

The major types of Hirschsprung disease are

 short-segment Hirschsprung disease, in which nerve cells are missing from

the rectum and sometimes the lower part of the colon, called the sigmoid colon.
 long-segment Hirschsprung disease, in which nerve cells are missing from the
rectum and a longer part of the colon than in short-segment Hirschsprung disease, but
nerve cells are present in at least part of the colon.
 total colonic Hirschsprung disease, in which nerve cells are missing from the rectum
and the entire colon, but are present in the end of the small intestine.
 small intestinal Hirschsprung disease, in which nerve cells are missing from the
rectum, colon, and the end of the small intestine, but are present in the rest of the
small intestine.
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 total intestinal Hirschsprung disease, in which nerve cells are missing from the
rectum, colon, and all, or nearly all, of the small intestine.

f. Diagnostic Procedures
Biopsy: During a rectal biopsy, your provider removes a small piece of rectal tissue.
Your provider examines the tissue under a microscope to check for nerve cells. In infants,
providers use a suction device to remove the tissue. This procedure isn’t painful and doesn’t
require anesthesia.
X-ray: Abdominal X-rays can show a bowel blockage.
Anorectal manometry: An anorectal manometry uses external pressure sensors and an
internal balloon device. It gauges how well your child’s rectum and anus get rid of poop. Your
child may be sedated during this test.
Barium enema X-ray: While your child is under sedation, a provider inserts a catheter
(thin tube) into your child’s anus. The catheter fills the intestine with barium, a safe, white liquid.
A technician takes X-rays as the barium travels through the intestine. A barium enema X-ray is a
type of lower gastrointestinal (GI) exam. It shows bowel obstructions and narrowed intestines.
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V. Pathophysiology

HIRSCHSPRUNG DISEASE

Predisposing factors
Common in male
Genetics
Family History

Intestines
(Sigmoid
Colon)

Serosa Smooth Muscles Submuscosa Mucosa Lumen

Plexus
DISRUPTED
Genes needed for
migration and
development of
Auerbach’s Plexus Meissner’s Plexus nerve fibers
(Myenteric Plexus) (Submucous Plexus)

No Peristalsis
Causes smooth Helps control blood RET EDNRB
muscle to relax flow, absorption and
secretion Loss of appetite
RET gene are Gene that
Constipation the most codes
common proteins that
Abdominal known connect the
distention genetic cause ganglions to
of the digestive
Hirschsprung tract
Megacolon disease

RET gene are

the most
common
known
genetic cause
of
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VI. Management
a. Medical Management
Initial therapy. If a child with Hirschsprung disease has symptoms and signs of a high-
grade intestinal obstruction, initial therapy should include intravenous hydration, withholding of
enteral intake, and intestinal and gastric decompression.
Decompression. Decompression can be accomplished through placement of a
nasogastric tube and either digital rectal examination or normal saline rectal irrigations 3-4 times
daily.
Diet. A special diet is not required; however, preoperatively and in the early
postoperative period, infants on a nonconstipated regimen, such as breast milk, are more easily
managed.
b. Surgical Management
Pull-through procedure

During a pull-through procedure, a surgeon removes the part of the large intestine that is
missing nerve cells. The surgeon then connects the healthy part of the large intestine to the anus.
Surgeons may perform the pull-through procedure as either laparoscopic or open surgery.
During laparoscopic surgery, surgeons make small cuts in the abdomen and insert a laparoscope
and tools to perform the operation. For open surgery, surgeons make a larger cut to open the
abdomen. In some cases, surgeons may perform the pull-through procedure by inserting tools
through the anus instead of making cuts in the abdomen.
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The three common pull-through procedures for Hirschsprung disease. In the Swenson
procedure, a radial full-thickness resection is begun in the aganglionic rectum just proximal to
the dentate line, and the aganglionic segment and transition zone are pulled through the anus and
resected. The transected end of ganglionic bowel proximal to the aganglionic segment is sutured
end to end to the terminal rectum. In the Soave procedure, the initial radial incision is made
through the mucosa, and the dissection is extended proximally in the submucosal plane for
several centimeters before dividing the muscularis propria. As a consequence, the muscularis
propria of the distal rectum is retained as a “cuff” (c), through which the ganglionic bowel is
drawn inferiorly and attached just above the dentate line. In the Duhamel procedure, the
pullthrough is performed along the posterior aspect of the aganglionic rectum, and ganglionic
bowel is attached in a side-to-side manner to the posterior rectum. The anterior rectum remains
as an aganglionic pouch.
Ostomy surgery
Doctors may recommend that children have ostomy surgery before having a pull-through
procedure, in some cases. For example, children may need ostomy surgery if they have
complications, such as severe Hirschsprung-associated enterocolitis, megacolon, or perforation.
Ostomy surgery allows a child’s health to improve and intestine to heal before pull-through
surgery. A child may also need ostomy surgery if the entire large intestine is affected by
Hirschsprung disease.
During ostomy surgery, surgeons create a stoma on a child’s abdomen and connect the
stoma to the large or small intestine. After ostomy surgery, waste will leave the child’s body
through the stoma.
The stoma is usually temporary. In most cases, surgeons can later close the stoma and
connect the healthy part of the intestine to the anus. Waste will move through the intestines, and
stool will pass through the anus again.
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c. Nursing Management
Nursing Management
Pre-Operative Care
 Assessement of complete history
 Nurse taught about giving isotonic enema, suppositories and stool softeners
 Low residue diet must be given to the child
 Monitor vital signs and abdominal girth of child
 Keep the child in semi-fowlers position
 Withhold oral feeds & Nasogastric aspiration done on the night before the surgery.
Post-Operative Care
 Monitor vital signs, observe abdominal bleeding
 Place the child in comfortable position according to the physician order
 Child is NPO, so administered IV fluids as ordered
 Monitor bowel sounds
 Colostomy is done which includes the following:
 Observe for stoma for its color (reddish-pink color)
 Observe for bleeding, purulent drainage, edema
 Provide bland diet
 Apply zinc oxide ointment on skin around stoma.
 Frequently empty the collecting bag
 Keep the colostomy clean and dry
 Educate parents about colostomy care
 Encourage and support the family during this stressful times
d. Discharge Planning

Colostomy care. Give careful attention to the area around the colostomy; record and report
redness, irritation, and rashy appearances of the skin around the stoma; prepare the skin with
skin-toughening preparations that strengthen it and provide better adhesion of the appliance.

High-fiber foods. If your child eats solid foods, include high-fiber foods. Offer whole grains,
fruits and vegetables and limit white bread and other low-fiber foods. Because a sudden increase
in high-fiber foods can worsen constipation at first, add high-fiber foods to your child's diet
slowly.
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Increase fluids. Encourage your child to drink more water. If a portion or all of your child's
colon was removed, your child may have trouble absorbing enough water. Drinking more water
can help your child stay hydrated, which may help ease constipation.

Laxatives (only as directed by your child's doctor). If your child doesn't respond to or can't
tolerate increased fiber, water or physical activity, certain laxatives — medications to encourage
bowel movements — might help relieve constipation. Ask the doctor whether you should give
your child laxatives, how often you should do so, and about the risks and benefits.

Daily physical exercises. This helps to promote regular bowel movements.

VII. Nursing Care Plan
Assessment Diagnosis Planning Intervention Rationale Evaluation
Subjective: Constipati Short term Independent: Short term
“Hindi on related goal: Determine stool Assists in identifying goal:
matae ang to After 10 color, consistency, causative or After 10
anak ko 3 decreased days, the frequency and contributing factors and days, the
days na” as bowel patient will amount. appropriate patient was
verbalized motility. experience interventions able to
by the passage of experience
mother. stool that Auscultate bowel Bowels sounds are passage of
will sounds generally decreased in stool that
Objective: improve constipation. will
Altered bow onwards. improve
el sounds. Encourage Sufficient fluid intake onwards.
Long term increased fluid is necessary for the
Reports of goal: intake of 2500 – bowel to absorb Long term
abdominal After 3 3000 ml/day sufficient amounts of goal:
pain or months, the within cardiac liquid to promote After 3
cramping. patient will tolerance. proper stool months, the
experience consistency. patient was
 V/S taken a regular able to
s follows: bowel Recommend Decrease gastric experience
BP: 120/80 movements Avoiding gas- distress and abdominal regular
T: 37.0 . forming foods distension. bowel
P: 112 such as nuts, peas movements.
R: 18 and spicy foods.

Instruct client on a Fiber absorbs water,

high-fiber diet, as which adds bulk and
appropriate. softness to the stool and
speeds up passage
through the intestines.
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Dependent:
Discuss use Facilitates defecation
of stool softeners, when constipation is
mild stimulants, present. After 8 hours
bulk forming of nursing
laxatives or interventions, the client
enemas as was able to establish or
indicated. Monitor return to normal
effectiveness patterns of bowel
functioning.

Collaborative:

Assessment Diagnosis Planning Intervention Rationale Evaluation

Subjective: Short term Independent: Short term
goal: goal:
Objective: Dependent:
Long term Long term
goal: Collaborative: goal:

VIII. Drug Studies

a. Bisacodyl
Name of Generic Dosage Action / Shape & Color or Nursing
Medication name Contraindication / the Medication Implications /
(Brand Side Effects CardWardtoBedbe# Used Nursing
name) 3-29-2023 Reponsibilities
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Dulcolax Bisacodyl 6-12yo: Action: Patient X Follow the 12

Bisacodyl 5mg once
5mg Laxative a day per orem rights in
once a 8am
administering
day PO Contraindication: Jaimie La Peña medication.
before In intestinal WUP SN 23
3-29-23
CI
signature
breakfast obstruction and in Inform the
case of any patient and
undiagnosed acute guardian about
or persistent the possible
abdominal side effects.
symptoms
Educate client
Side Effects: and guardian
Stomach/abdominal to promote
pain or cramping, understanding
nausea, diarrhea, or and
weakness compliance.

b. Ampicillin
Name of Generic Dosage Action / Shape & Color Nursing
Medication name Contraindication / or the Implications /
(Brand Side Effects Medication Nursing
name) Card Ward
to3-29-2023
be Used
Bed #
Reponsibilities
Principen Ampicillin 50mg/kg Action: Patient X
Follow the 12
IV q6 Antibiotic Ampicillin 50mg/kg
every 6 hours intravenously
rights in
12mn-6am-12nn-6pm
administering
Contraindication: Jaimie La Peña
medication.
Patients who have WUP SN 23 CI
3-29-23 signature
allergy to this Inform the
medication patient and
guardian about
Side Effects: the possible
Nausea, vomiting, side effects.
diarrhea, and mild
rashes Educate client
and guardian to
promote
understanding
and
compliance.
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Monitor patient
for
hypersensitivity
reaction
especially if
drug istaken
previously.
Do not use
anti-diarrhea
medication if
symptoms
occur because
this can only
worsen the side
effects.