College of Nursing

Silliman University
Dumaguete City

Resource Unit on Care of Patient with Hirschsprung’s Disease

Submitted to:
Asst. Prof. Kathleah Caluscusan

Submitted by:
Lasola, Genmarie C.
Isabelo, Micah Ella B.
 College of Nursing
Silliman University
Dumaguete City

Vision:
A leading Christian institution committed to total human development for the wellbeing of society and environment.

Mission:
1. Infuse into the academic learning the Christian faith anchored on the gospel of Jesus Christ; provide an environment where Christian fellowship
and relationship can be nurture and promoted.
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3. Instill in all members of the University community an enlightened social consciousness and a deep sense of justice and compassion
4. Promote unity among people and contribute to national development.
Topic: Care for Patient with Hirschsprung Disease

Time allotment: 1 hour

Central Objective: After 1 hour of our discussion, the learners would have sufficient knowledge as how to provide appropriate care to patients with Hirschsprung’s disease.
Topic description: This resource unit contains the objectives, definition, clinical manifestations, nursing and therapeutic management related to the concept in dealing with
patients having Hirschsprung’s disease.

OBJECTIVES CONTENT T-L T.A. REFERENCES EVALUATION

ACTIVITIES METHOD
By actively
participating in the I. Care of Patient with Hirschsprung’s disease Outcome
learning activities, the Evaluation:
learners will:
A. Contextual definitions: Wong, D. (1999). Whaley Assessment through
 Correctly & Wong's Nursing Care of written examination (15
Hirschsprung’s disease is a congenital anomaly points) of learner’s
define Lecture 3 min Infants and Children (6th understanding and own
Hirschsprung’s that results in mechanical obstruction from Discussion ed.). St. Louis: Mosby. thoughts on:
disease with inadequate motility of part of the intestines. It Using Ball, J., & Bindler, R.
their own accounts for about one fourth of all cases of PowerPoint (2008).  Definition of
words. neonatal intestinal obstruction. It may not be presentation Pediatric Nursing: Caring the disease
diagnosed until later in infancy or childhood. for Children (4th ed.).
 The clinical
The incidence is 1 in 5000 live birth. It is four Upper Saddle River, N.J.: manifestations
times more common in males than in females Pearson/Prentice Hall. of
and follows a familial pattern in a small number Hockenberry, M., Wilson, hirschsprung’s
of cases. Hirschprung disease is associated with D., & Rodgers, C. disease in
other anomalies, such as Down syndrome. infancy and
Wong's Essentials of
childhood
Depending on its presenation, it may be an Pediatric Nursing (7th
acute, life- threatening condition or a chronic ed.).  Different types
disorder. Klossner, N., & Hatfield, of diagnostic
N. (2010). tests in
B. Pathophysiology determining the
 Understand Introductory maternity &
signs and
fully the pediatric nursing. symptoms of
physiological The term congenital ganglionic megacolon Lecture 10 min Philadelphia: Wolters hirschsprung’s
describes the primary defect, which is the
 process of absence of autonomic parasympathetic ganglion Discussion Kluwer/Lippincott disease
Hirschprung’s cells in the submucosal (Meissner) and Using Williams & Wilkins
disease. myenteric (Auerbach) plexuses in one or more PowerPoint Health.  Therapeutic
segments of the colon. Lack of innervation presentation management on
produces the functional defect (i.e., absence of the client with
propulsive movements [peristalsis]), which hirschsprung’s
causes accumulation of intestinal contents and disease
bowel distention proximal to the defect
 How to manage
(megacolon). In addition, failure of the internal complications
anal sphincter to relax contributes to clinical that may occur
manifestations of obstruction, be- cause it during the
prevents evacuation of solids, liquids, and gas. intraoperative
Hirschsprung disease results from failure of and postop
phase of the
craniocaudal migration of ganglion cell surgical
precursors along the GI tract between the fifth treatment of the
and twelfth weeks of gestation. The aganglionic disease
segment almost always includes the rectum and
some portion of the distal colon, but the entire  The common
drug prescribe
colon or part of the small intestine may be on patients with
involved. Rarely, skip segments or total hirschsprung’s
intestinal aganglionosis may occur. Intestinal disease
distention and ischemia may occur as a result of
distention of the bowel wall, which contributes  Nursing
responisibilities
to the development of enterocolitis on giving care
(inflammation of the small bowel and colon), to patient with
the leading cause of death in children with hirschsprung’s
Hirschsprung disease. disease

 Identify the C. Clinical Manifestations

signs and
symptoms of Clinical manifestations vary according to the age Lecture 5 min Wong, D. (1999). Whaley
Hirschprungs when symptoms are recognized, the length of the Discussion & Wong's Nursing Care of
disease in affected bowel, and the occurrence of Using Infants and Children (6th
 newborns, complications, such as enterocolitis. In the PowerPoint ed.). St. Louis: Mosby.
infancy, and newborn the primary signs and symptoms are presentation
childhood. failure to pass meconium within 24 to 48 hours
after birth, food refusal, vomiting, abdominal
distention, and intestinal obstruction.

a) During infancy inadequate weight gain,

constipation, abdominal distention, and
episodes of diarrhea and vomiting are likely
to occur. Bloody diarrhea, fever, and severe
lethargy are ominous signs because they
often signify the presence of enterocolitis,
which greatly increases the risk of fatality.
Enterocolitis may also be present without
diarrhea and is first evidenced by
unexplained fever and poor feeding.

b) During childhood the symptoms become

chronic and include constipation, passage of
ribbon-like, foul-smelling stools, and
abdominal distention. Fecal masses may be
palpable. Fecal soiling is uncommon, but
fecal impactions recur frequently. The child
usually has a poor appetite and poor
growth.

 Describe the
different types D. Diagnostic Evaluation
of diagnostic Lecture 5 min Tazbir, J., & Keresztes, P.
tests used to Tests used to help diagnose Hirschsprung’s Discussion (2008). Atlas of medical-
diagnose disease may include: Using surgical nursing. Clifton
and/or ● Abdominal x-ray PowerPoint Park, NY: Thomson
determine the ● Anal manometry presentation Delmar Learning.
extent of ● Barium enema Wong, D. (1999). Whaley
Hirschprungs ● Rectal biopsy & Wong's Nursing Care of
disease. Infants and Children (6th
In the neonate the diagnosis is suspected ed.). St. Louis: Mosby.
on the basis of clinical signs of intestinal
obstruction or failure to pass meconium.
In infants and children the history is an
important part of diagnosis and typically details
a chronic pattern of constipation. On
examination the rectum is empty of feces, the
internal sphincter is tight, and leakage of liquid
stool and accumulated gas may occur if the
aganglionic segment is short. A barium enema
often demonstrates the transition zone between
the dilated proximal colon (megacolon) and the
aganglionic distal segment. However, this
typical megacolon and narrow distal segment
may not develop until the age of 2 months or
later in some affected children. To confirm the
diagnosis, rectal biopsy is performed either
surgically to obtain a full-thickness biopsy
specimen or by suction biopsy for histologic
evidence of the absence of ganglion cells. A
noninvasive procedure that may be used is
anorectal manometry, in which a catheter with
a balloon attached is inserted into the rectum.
The test records the reflex pressure response of
the internal anal sphincter to distention of the
balloon. A normal response is relaxation of the
internal sphincter followed by a contraction of
the external sphincter. In Hirschsprung disease
the external sphincter contracts normally but the
internal sphincter fails to relax.
 Analyze the E. Therapeutic Management
medical
management A. Medical Management
and the many Lecture 15 min Karch, A. (2008). 2008
surgical Metronidazole (Flagyl) Discussion Lippincott's Nursing Drug
managements PREGNANCY CATEGORY B Using Guide. Philadelphia, Pa.:
of PowerPoint Wolters Kluwer/Lippincott
Hirschprungs Drug classes : presentation Williams & Wilkins.
disease.  Antibiotic Vallerand, A. (2014).
 Antibacterial Davis's Drug Guide for
 Amebicide Nurses. F.A. Davis
 Antiprotozoal Company.

Pharmacodynamics :
Bactericidal: inhibits DNA synthesis in specific
(obligate) anaerobes, causing antiprotozoal-
trichomonacidal, amebicidal; biochemical
mechanism of action is not known.

Indications:
• Acute infection with susceptible anaerobic
bacteria
• Acute intestinal amebiasis
• Amebic liver abscess
• Trichomoniasis (acute and partners of
patients with acute infection)
• Preoperative, intraoperative, postoperative
prophylaxis for patients undergoing
colorectal surgery
 • Topical application in the treatment of
inflammatory papules, pustules, and
erythema of rosacea
• Unlabelled uses: prophylaxis for patients
undergoing gynecologic, abdominal
surgery; hepatic encephalopathy;
Crohn's disease; antibiotic-associated
pseudomembraenous colitis; treatment of
GardnereIIa vaginalis, giardiasis (use
recommended by the CBC)

Contraindications and cautions:

• Contraindicated with hypersensitivity to
metronidazole; pregnancy (do not
for trichomoniasis in first trimester).
• Use cautiously with CNS diseases, hepatic
disease, candidiasis (moniliasis),
blood dyscrasias, lactation.

Pharmacokinetics:

Route Onset Peak

Oral Varies 1-2 hr

IV Rapid 1-2 hr

Topical Generally no systemic absorption

Metabolism: Hepatic, T1/2= 6-8 hr

Distribution: Crosses placenta; enters breast
milk
Excretion: Urine and feces

Adverse effects:
CNS: Headache, dizziness, ataxia, vertigo.
incoordination, insomnia, seizure;
peripheral neuropathy, fatigue
GI: unpleasant metallic taste, anorexia,
nausea, vomiting, diarrhea, Gl
upset, cramps
GU: incontinence, dysuria, darkening of the
urine
Local: Thrombophlebitis (IV); redness,
burning, and skin irritation (topical)
Other: severe, disulfiram-like interaction
with alcohol, candidiasis (superinfection)

Nursing considerations:
Assessment
• History: CNS or hepatic disease,
candidiasis (moniliasis), blood
dyscrasias; pregnancy; lactation
• Physical: Reflexes, affect, skin
lesions, color (with topical
application); abdominal
exam, liver palpation; urinalysis,
CBC liver function tests
Interventions
• Avoid use unless necessary.
Metronidazole is carcinogenic in
some rodents.
• Administer oral doses with food
 • Reduce dosage in hepatic disease.
Teaching points
• Take full course of drug therapy;
take drug with food if GI upset
occurs,
• side effects may dry mouth with
strange metallic taste (frequent
mouth care, sucking sugarless
candies may help); nausea,
vomiting, diarrhea (small,
frequent meals may help).
• Do not drink alcohol (beverages or
preparations containing alcohol,
cough syrups); severe reactions may
occur
• Be aware that your urine may
appear dark; this is expected,
• Report GI upset, dizziness, unusual
fatigue or weakness, fever, chills.

B. Surgical Management Wong, D. (1999). Whaley

& Wong's Nursing Care of
The vast majority of children with Infants and Children (6th
Hirschsprung’s disease require surgery ed.). St. Louis: Mosby.
rather than medical therapy with frequent Ball, J., & Bindler, R.
enemas. Once the child is stabilized with (2008).
fluid and electrolyte replacement, if needed,
surgery is performed with a high rate of
success. The surgical management consists
primarily of the removal of the aganglionic
portion of the bowel in order to relieve
obstruction, restore normal motility, and
preserve the function of the external anal
sphincter. In most cases this is
accomplished in two stages.

B.1) Temporary Ostomy (First Surgery)

-An ostomy is a surgical procedure

in which an opening is made to
allow the passage of intestinal
contents from the bowel to an
incision or stoma.
-First, a temporary ostomy is created
proximal to the aganglionic
segment, which relieves obstruction
and allows the normally innervated
dilated bowel return to its normal
size.

B.2) Second surgery

Following the initial surgery a

second complete, corrective surgery
is performed, usually when the child
weighs approximately 9 kg (20
pounds) (Kirschner, 1996). There
are several definitive operations that
can be performed, including the
Swenson, Duhamel, Boley, and
Soave procedures. The Soave
endorectal pull- through procedure
is often performed and consists of
pulling the end of the normal bowel
through the muscular sleeve of the
 rectum, from which the aganglionic
mucosa has been removed. The
ostomy is usually closed at the time
of the final, definitive surgery.
Simpler operations, such as an
anorectal myomectomy, may be
indicated in very-short- segment
disease.

B.3) Complications Schleef, J and Olenik, D,

Complications in
B.3.1) Intra operative Hirschsprung’s Disease.
(2013). Retrieved from
As in any other surgical procedure, https://www.
intraoperative complications might researchgate.net/publication
occur and can be responsible for /236619297_Complications
tragic post-op courses and _in_Hirschsprungs_Disease
reoperations. Many of these are
general complications like bleeding,
injury to other organs or not
recognized damage to the bowel
remnant, left in place. All minimal-
invasive-techniques, as well as the
transanal approach have the
advantage of reduced tissue trauma,
less stress due to surgery and better
cosmetically results. But it should
be emphasized that not all patients
are suitable for these procedures.
Another important intraoperative
complication is the twisting of the
bowel, performing a pullthrough.
 B.3.2) Postoperative

In the post op period all general

complications after abdominal
surgery can occur (haematoma,
infection, bleeding). Post- op bowel
occlusion seems to be less frequent
after laparoscopic and trans anal
pullthtrough surgery. In some cases,
a part of the small bowel can slip
under the mesentery of the distal
colon and might cause occlusion.
This problem can be resolved by
stitches, anchoring the neorectum to
the presacral fascia structures.
Perianal excoriation can be seen in
at least one third of HD parients
after surgery. Using barrier creams
can resolve these problems. In some
instances it might be helpful to
reduce the amount of bile salts using
cholestyramine.

B.4) Prognosis Wong, D. (1999). Whaley

Most children with Hirschsprung
disease require surgery rather than
medical therapy. Once the child is
stabilized with fluid and electrolyte
replacement, if needed, the
temporary colostomy is performed
 Explain the with a high rate of success.
& Wong's Nursing Care of
Infants and Children (6th
ed.). St. Louis: Mosby.
Ball, J., & Bindler, R.
(2008).
nursing Following the later pull- through
considerations procedure, anal stricture and
needed in the incontinence are potential
care of a complications that may occur and
patient with require further therapy, including
Hirschprung’s dilations or bowel-retraining
disease that therapy.
will or have
undergone E.) Nursing Considerations Lecture 10 min Wong, D. (1999). Whaley
medical and/or Discussion & Wong's Nursing Care of
surgical Many of the nursing concerns depend on the Using Infants and Children (6th
management of child’s age and the type of treatment. Nursing PowerPoint ed.). St. Louis: Mosby.
the disease. observation of passage of meconium and bowel presentation Ball, J., & Bindler, R.
patterns in the neonatal period is an important (2008).
factor in early diagnosis. If the disorder is Pediatric Nursing: Caring
diagnosed during the neonatal period, the main for Children (4th ed.).
objectives are helping the parents adjust to the Upper Saddle River, N.J.:
congenital disorder in their child, fostering infant- Pearson/Prentice Hall.
parent bonding, preparing them for the Hockenberry, M., Wilson,
medical/surgical intervention, preparing them in D., & Rodgers, C. Wong's
caring for the colostomy after discharge. Essentials of Pediatric
When the disorder is not discovered during this Nursing (7th ed.).
period the nurse can facilitate establishing a Klossner, N., & Hatfield,
diagnosis by carefully listening to the history, with N. (2010).
a special emphasis on bowel habits. In Introductory maternity &
Hirschsprung disease, several areas must be pediatric nursing.
investigated: (1) frequency of bowel movement; (2) Philadelphia: Wolters
character of stools, particularly ribbonlike and foul- Kluwer/Lippincott
smelling stools; and (3) onset of constipation, Williams & Wilkins
especially if present since birth. Other clues in the Health.
history and physical examination include poor
feeding habits, fussiness and irritability, distended
abdomen, and signs of undernutrition, such as thin
extremities, pallor, muscle weakness, and fatigue.

A) Surgical Therapy

A.1.1) Preoperative Care

Much of the child’s preoperative

care depends on the age and clinical
conditions. A child who is
malnourished may not be able to
withstand surgery until the physical
status improves. Often this involves
symptomatic treatment with enemas;
a low-fiber, high-calorie, and high-
protein diet; and in severe situations,
the use of total parenteral nutrition
(TPN).

A.1.2) Postoperative Care

Postoperative care following a

colostomy or pull- through
procedure is similar to that
following abdominal surgery. The
infant or child should have nothing
by mouth and will often have an NG
tube to suction. To prevent
contamination of the abdominal
wound with urine in the infant, the
diaper should be pinned below the
dressing. Intravenous fluid are
 monitored to maintain adequate
hydration and electrolyte balance.
And abdominal assessment,
including monitoring of return of
bowel sounds and passage of stool,
will indicate when oral feeding can
be initiated. Following a colostomy
procedure, ostomy care is an
important nursing responsibility.
Ongoing education of the older child
and caregivers regarding ostomy
care will begin with preparation for
their discharge home.

A.1.3) Home Care

Postoperatively, parents need

instruction concerning colostomy
care at home, including skin care,
emptying and changing the ostomy
appliance, and monitoring for
problems. During the early
postoperative period, including
parents and the older child in
dressing changes can enhance
teaching of colostomy care when an
appliance is fitted and promote
gradual acceptance of the body
change,

B) Medical Therapy

The goals of medical care are to maintain Wong, D. (1999). Whaley

normal fluid and electrolyte balance, to & Wong's Nursing Care of
minimize bowel distension and prevent Infants and Children (6th
perforation, and to manage complications. ed.). St. Louis: Mosby.
Intravenous fluid resuscitation and Ball, J., & Bindler, R.
maintenance, nasogastric decompression, (2008).
and administration of intravenous
antibiotics (as indicated) remain the
cornerstones of initial medical management.

Colonic lavage, consisting of mechanical Nursing Implications for

irrigation with a large-bore rectal tube and Diagnostic Tests. (2017).
large volumes of irrigant, may be required. Retrieved from
http://wps.prenhall.com/
Intravenous administration of balanced wps/media/objects/
salt solutions may help prevent electrolyte 737/755395/barium_
imbalances. enema.pdf
Hirschsprung Disease.
(2017).
Misc.medscape.com.
Retrieved 18 June 2017,
from http://misc.medscape.
com/pi/iphone/med
scapeapp/html/A178
493-business.html
Gil Wayne, R. (2017).