QUESTIONS:

1. What causes Hirschprung’s Disease?

 Hirschsprung's disease is caused when the muscles of the large intestine cannot contract
and relax as required. This happens due to problems in the embryonic development
when the nerves that control this movement stop growing towards the end of the
bowel. The following factors are associated with increased risk of development of the
disease
 Genetic mutations: mutations in certain genes such as the RET proto oncogene are
associated with this condition
 Family history: Individuals who have affected Family members are more likely to
develop this disease
 Associated syndromes: Hirschsprung's disease is sometimes present as part of a
syndrome such as Down syndrome, Waardenburg syndrome or multiple endocrine
neoplasia

2. What are the clinical manifestations of Hirschsprung disease in an

infant?
Swollen belly. Vomiting, including vomiting a green or brown substance. Constipation or gas,
which might make a newborn fussy. Diarrhea.

3. What diagnostic procedure that confirms Hirschprung’s Disease?

A surgical consultation was placed, and the patient underwent a full-thickness
rectal biopsy that revealed absence of ganglion cells and abnormal
acethylcholinesterase staining confirming the diagnosis of Hirschsprung’s
Disease(HD).

 A contrast enema: It’s also called a barium enema, after the element in

the dye often used to coat and highlight the inside of the body’s organs.
Your child is placed face-down on a table while the dye is inserted via a
tube from the anus into the intestines. No anesthesia (a medication to
numb or block pain) is necessary. The dye allows the doctor to see
problem areas on the X-rays.
  An abdominal X-ray: This is a standard X-ray, which the technician may
take from several angles. Your doctor will be able to see whether
something is blocking the intestines.
 A biopsy: Your doctor will take a small sample of tissue from your child’s
rectum. The tissue will be looked at for signs of Hirschsprung’s.
Depending on the age and size of your child, the doctor may use
anesthesia.
 Anorectal manometry: This test inflates a small balloon inside the rectum
to see whether the muscles of the area respond. This test is done only on
older children.

4. What are the nursing responsibilities to patient with Hirschprung’s

Disease?
The major nursing care planning goals for patients with Hirschsprung Disease are: 

 Maintaining skin integrity. 

 Promoting comfort. 
 Maintaining fluid balance.
 Maintaning moist, clean nasal and oral membranes.
 Reducing caregiver anxiety.

5. What are the different types of Hirschsprung disease?Explain your

answer.
short-segment disease - nerve cells are missing from only the last segment of the large
intestine (colon). This type is most common, occurring in approximately 80 percent of people
with Hirschsprung disease. For unknown reasons, short-segment disease is four times more
common in men than in women.
Long-segment disease- occurs when nerve cells are missing from most of the large intestine
and is the more severe type. Long-segment disease is found in approximately 20 percent of
people with Hirschsprung disease and affects men and women equally.

6. Discuss the pathophysiology of Hirschprung’s Disease?

Hirschsprung disease results from the absence of enteric neurons within the myenteric and
submucosal plexus of the rectum and/or colon. Enteric neurons are derived from the neural
crest and migrate caudally with the vagal nerve fibers along the intestine.
 7. Formulate a pre-operative Nursing Diagnosis.
- Risks for Growth and Development related to congenital disorder.
- Altered bowel elimination related to abdominal distention
- Imbalanced nutrition related to less than body requirements

8. Identify at least 2 nursing problem and make a Nursing Care Plan.

Defining Nursing Outcome Nursing Rationale Evaluation
characteristics diagnosis identification interventions

Subjective: >acute pain >After 8 hours Independent: The following

related to of nursing nursing
‘’my child failure to 1.establish 1. to gain trust
abdominal intervention, interventions are
pass meconium rapport and and cooperation.
distention, the patient will met as evidenced
stained for good working
hypoactive able to by:
24hours’’ as relationship to
bowel sounds, demonstrate
verbalized by the the mother. 1. goals were met as
reducible pain is relieved
mother. evidenced by the
umbilical or controlled as
mother
hernia and evidenced by
2. Assess pain 2.The infant is communicate well
surgical relaxation skills,
using facial not able to with the nurse.
response as and other
evidenced by methods to expression verbalize pain 2. goal was met as
physical promote scale that is but still, there evidenced by the
Objective: examination. comfort. appropriate to are pain clues nurse was able to
the age. that can be
 presented assess and evaluate
recognize by the results of pain
to the
Rationale: > After 8 hours nurse. interventions.
emergency
room with of nursing
Unpleasant 3. goal was met as
complaints intervention, 3. Monitor skin
sensory and 3.which are evidenced by the
of failure to the patient able color and
emotional usually altered in nurse was able to
pass to report pain is temperature
experience acute pain monitor normal skin
meconium relieved or and vital signs
associated color and
after 24 controlled as (e.g., heart
with actual or temperature and
evidenced by
 hours potential absence of rate, blood vital signs.
tissue damage, intermittent pressure,
 Temperatur 4.to comfort the 4.goals was met as
or described in crying. respirations)
e = 37.2ºC child. evidenced by the
terms of such
4. Encourage mother was able to
 PR=150BPM damage;
presence of comfort child during
sudden or
 RR= 46BPM parent during the painful
slow onset of
painful procedure.
 Wgt= 3.2kgs any intensity 5. Observations
procedures
from mild to may not be 5.goals was met as
severe and 5. Observe congruent with evidenced by the
with a nonverbal cues verbal reports or child was able to
duration of and pain may be only show non-verbal
less than 3 behaviors (e.g., indicator present cues and pain
months. facial when client is behaviors.
expression; cool unable to
finger tips/toes, verbalize.
which can
mean 6. reduces muscle
constricted tension or 6. goals was met as
blood vessels). guarding, which evidenced by
may help patient was able to
6. move patient minimize pain of reduce muscle
slowly and movement. tension and
deliberately. minimize pain.
7. promotes
relaxation and 7. goals was met as
pain evidenced by the
management. patient was able to
7. Provided feel relax and
comfort comfort.
measures like
calming the
infant after a
stressful
procedure,
gently pat or
massage the
infant, talking
in a soothing
voice, hold your
8.To facilitate
infant with as 8. goals was met as
comfort, sleep,
much skin-to- evidenced by the
and relaxation
skin contact as patient was able to
possible. sleep well.
 8. Provided
quiet
environment by
diminishing the
noise, stimulant
and less activity 1. Provides
in the bedside. opportunity to
1. goal was met as
modify pain
Dependent: evidenced by the
management
nurse was able to
regimen and
Interdependent identify specific s&s
allows for timely
: and changes in pain
intervention for
characteristics of
1. Identify developing
the patient.
specific complications.
signs/symptom 2.goal was met as
2. assist client to
s and changes evidenced by the
explore methods
in pain nurse was able to
for
characteristics assist with and
alleviation/contr
requiring review results of
ol of pain.
medical follow- laboratory tests and
up. diagnostic studies of
the patient.

2. assist with
and review
results of
laboratory tests
and diagnostic
studies
depending on
results of
history and
physical
examination.

Defining Nursing Outcome Nursing Rationale Evaluation

characteristics diagnosis identification interventions

Subjective: >risk for Long term: Independent:

infection
‘’my child failure to >After 3 days 1. establish 1. to gain trust 1. goal was met
related to the
pass meconium of nursing rapport and good and cooperation. as evidenced by
post-surgical
stained for intervention, working mother
procedure.
24hours’’ as the patient relationship to communicate
verbalized by the will be able to the mother. well with the
Rationale:
mother prevent the nurse.
A break in the risk for
Objective: 2. goals met as
first line of infection. 2. monitor vital
2. to determine if evidenced by
 presented defense by the signs.
there has been normal vital signs
to the body, the skin, (temperature)
systemic of the patient
emergency would Short term:
infection
room with promote the
After 6 hours occurring inside
complaints entrance of
of nursing the body.
of failure to microorganism
intervention, 3. assess
pass which can 3. Provides 3. goal was met
the patient changes of woun
meconium cause comparative as evidenced by
with the help d site or
after 24 infection at baseline for nurse was able to
of the mother depth, width,
hours wound site or future asses changes in
will be able color, smell,
even sepsis assessment and wound of the
 The neonate to: location,
through the promote timely patient.
was temperature,
body’s blood 1. perform nursing
scheduled texture, and
circulation if independently intervention and
for discharges
not treated proper wound revision of care
abdomino-
properly. care. plan" It also
pull-through
determines the
surgery. He after 3 days of 3. identify
risk or degree
undergoes 2 nursing interventions
of infection of th
stages of intervention that could
e wound.
surgery, the the patient prevent or 4. Maintain strict
1st stage is was able to reduce the asepsis for 4. Aseptic
temporary prevent risk of risk for dressing changes, technique
colostomy infection with infection. wound decreases the
and tube, the help of her care, intravenous chances of
mother. 4. achieve
followed by therapy, and transmitting or
timely wound 4. goal is met as
bowel catheter handling spreading
healing, free evidenced by the
repair when pathogens to or
from signs of patient able to
he reaches between
infection. achieve timely
the age of patients.
wound healing
12- 5. verbalize Interrupting the
and free from
18months of feelings or chain of
signs of infection.
age. understanding infection.
5.teach the patie
, recovery and nt’s mother how
 Temperatur 5. to promote
comfort. to do
e = 37.2ºC faster wound 5. goal is met as
proper wound healing and evidenced by the
 PR=150BPM caring. recovery. mother was able
 RR= 46BPM to do proper
wound healing to
 Wgt= 3.2kgs her child.

9. Discuss the pull-through-surgery.

- The goal of pull-through surgery is the removal of diseased section on your
child's intestine and then pull the healthy portion of this organ down to the anus.
In most cases, this procedure can be done with minimally invasive techniques in
a single operation. Surgeons typically perform the pull-through procedure when a
child is initially diagnosed with Hirschsprung's disease. The physician will first
take one or more tissue samples (biopsy) of your child's bowels to identify the
areas of the intestine that lack ganglion cells. The surgeon will then remove
these sections of diseased intestine, either through a large incision in your child's
abdomen (open surgery) or by inserting a thin, lighted tube with a camera on it
(laparoscope) through a smaller incision. In some cases, the pull-through
procedure can be performed entirely through the child's anus, which doesn't
leave any scars.

After removing the portion of intestine affected by Hirschsprung's disease, the

surgeon will then attach the end of the remaining, healthy intestine to the child's
anus.

10. What are the health education and health maintenance after
discharged?

 Skin Care
- It is important to protect the skin around the anus at all times to prevent
irritation, using a cream called "butt balm." Do not stop using the skin
protective products until your child is having fewer bowel movements,
usually after many weeks.
 Dressings
- If your baby 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. Your
child can bathe with the Steri-strips in place.
 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 your child is still uncomfortable,
call our office and we may prescribe something stronger.
 Follow-up Appointments
- If your child has a stoma, you will need to make an appointment to be
seen one after discharge. If your child has a primary surgery or stoma
closure, he or she will need to be seen in the Pediatric Surgery office
two weeks after the operation.
 Signs to Watch For:

o Temperature of 101° F or higher

o Incision is red
o Severe pain at the incision or the incision is painful to touch
o Fluid coming out of the incision
o Change in the number of bowel movements each day
o No bowel movement for one day
o Red rash around the anus that is not getting better

11. What are the expected outcome postoperatively?

 Constipation
There are three mechanisms for chronic post-operative constipation:
1. functional constipation;
2. neuropathy, or abnormal functioning of nerves above the
aganglionic segment; and
3. hypertensive anal sphincter, in which the sphincter doesn’t relax
normally, making it difficult to push stool past it.

 Colon Manometry
 -Colon manometry discriminates functional constipation from colon
neuropathy. Colon manometry is a simple test taking two or three
hours in most cases, but preparation is complicated.
 Fecal Incontinence
-Fecal incontinence has more than one mechanism in post-
operative Hirschsprung’s disease: high-amplitude propagating
contractions through the neorectum (newly created rectum), and
functional fecal retention.
 Abdominal Pain
-Abdominal pain may occur whenever the child senses an urge to
defecate, but chooses to tighten the sphincter and avoid a bowel
movement. Some children with a normal increase in colon
contractions after a meal perceive those contractions as a pain
rather than an urge to have a bowel movement. Over time the urge
to defecate may change to a pain perception, so that the child no
longer recognizes when it is time to have a bowel movement.

12. Explain Enterocolitis as one of the most common complication

of Hirschsprung’s Disease .
Enterocolitis is an inflammation that occurs in a person’s digestive tract.
The condition specifically affects the inner linings of both the small
intestine and the colon, causing several symptoms. It may be caused by
various infections, with bacteria, viruses, fungi, parasites, or other causes.
Common clinical manifestations of enterocolitis are frequent diarrheal
defecations, with or without nausea, vomiting, abdominal pain, fever,
chills, alteration of general condition. General manifestations are given by
the dissemination of the infectious agent or its toxins throughout the body,
or – most frequently – by significant losses of water and minerals, the
consequence of diarrhea and vomiting.

13. Define the following terms:

a.) BARIUM ENEMA- A barium enema is an X-ray exam that can detect changes or
abnormalities in the large intestine (colon). The procedure is also called a colon X-ray.
An enema is the injection of a liquid into your rectum through a small tube. In this
case, the liquid contains a metallic substance (barium) that coats the lining of the
colon. Normally, an X-ray produces a poor image of soft tissues, but the barium
coating results in a relatively clear silhouette of the colon.
b.) MANOMETRY- Manometry is measurement of pressure within various parts
of the gastrointestinal tract. It is done by passing a catheter containing solid-state
or liquid-filled pressure transducers through the mouth or anus into the lumen of
the organ to be studied. Manometry typically is done to evaluate motility
disorders in patients in whom structural lesions have been ruled out by other
studies. Manometry is used in the esophagus, stomach and duodenum, sphincter
of Oddi, and rectum. Aside from minor discomfort, complications are very rare.
Patients must have nothing by mouth (npo) after midnight.

c.) SWENSON PROCEDURE- The Swenson procedure was the original pull-
through procedure used to treat Hirschsprung disease. The aganglionic segment
is resected down to the sigmoid colon and rectum, and an oblique anastomosis is
performed between the normal colon and the low rectum.
d.) DUHAMEL PROCEDURE- The Duhamel procedure was first described as a
modification to the Swenson procedure. A retrorectal approach is used, and a
significant segment of aganglionic rectum is retained. The aganglionic bowel is
resected down to the rectum, and the rectum is oversewn. The proximal bowel is
then brought through the retrorectal space (between rectum and sacrum), and an
end-to-side anastomosis is performed with the remaining rectum.
e.) SOAVE PROCEDURE- The Soave procedure was introduced in the 1960s. The
mucosa and submucosa of the rectum are resected, and the ganglionic bowel is pulled
through the aganglionic muscular cuff of the rectum. The original operation did not
include a formal anastomosis, relying on scar tissue formation between the pull-
through segment and the surrounding aganglionic bowel. The procedure has
since been modified by Boley to include a primary anastomosis at the anus.