Placino HIRSCHPRUNG DX PDF

Manuel S.
Enverga University Foundation

Lucena City
Granted Autonomous Status
CHED CEB Res.076-2009
COLLEGE OF NURSING & ALLIED HEALTH SCIENCES
COLLEGE OF NURSING ALLIED HEALTH SCIENCES
In partial fulfillment of the requirements for

Related Learning Experience at
INP101 Intensive Nursing Practicum
NURSING CASE STUDY

HIRSCHPRUNG DISEASE
Submitted by:
Placino, Siena Kathleen V.
Submitted to:
Clinical Instructor
Helen Noime Rigos Guce, RN, MAN
Date Submitted:
May 5, 2020
Manuel S. Enverga University Foundation
Lucena City
I. BACKGROUND OF THE DISEASE

a. INTRODUCTION
i. OVERVIEW
ii. Hirschprung Disease is also termed as “Congenital Aganglionic Megacolon,” and
is defined as the congenital absence of or arrested development of
parasympathetic ganglion cells in the intestinal wall, usually in the distal colon.
This congenital lack of colorectal innervation inhibits peristaltic movement and
the affected portion of the intestine becomes spastic and contracted.
Mechanical obstruction results because of inadequate motility in an intestinal
segment. The internal rectal sphincter fails to relax, which prevents evacuation
of fecal material and gas, thereby causing severe abdominal distention and
constipation.
iii. The estimated incidence is 1 in 5000 live births. (MSD Manuals)
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Image above, pediatric hirschsprung

disease, from EMedicine: Medscape.
iv. A newborn who has Hirschsprung’s disease usually cannot have a bowel
movement in the days following birth. In mild cases, the condition may not be
detected until childhood. Surgery to bypass or remove the diseased part of the
colon is the treatment.
b. RISK FACTORS / CAUSES
During in-utero development of the baby,
nerve cells stop growing toward the end of a child’s
bowel causing Hirschsprung disease.
The etiology of aganglionosis is thought to be

the failure of migration of neuroblasts from the
neural crest. There is a significant genetic
component to this disorder and at least 12 different
genetic mutations are associated with Hirschsprung.
(MSD Manuals) Nerves in the colon control the
muscle contractions that move food through the
bowels. Without these nerves and corresponding
peristaltic contractions, stool stays stuck in the large
intestine and does not pass.
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According to Mayo Clinic, it is not presently clear what causes Hirschsprung’s disease. It sometimes
occurs in families and might, in some cases be associated with a genetic mutation. Factors that may
increase the risk of Hirschsprung’s disease include:
1. 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 children could be at risk.
2. Being male.
3. 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.
c. PATHOPHYSIOLOGY
d. SIGNS AND SYMPTOMS

i. Patients most commonly present early in life, but some do not present until
childhood or even adulthood.
ii. Assessment may reveal the following: (Saunders, 2017)
1. In newborns:
a. Failure to pass meconium stool
b. Refusal to suck
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c. Abdominal distention
d. Bile-stained vomitus
2. In children
a. Failure to gain weight and delayed growth
b. Abdominal distention
c. Vomiting
d. Constipation alternating with diarrhea
e. Ribbon-like and foul-smelling stools
e. COMPLICATIONS
i. In Hirschsprung’s disease, the intestinal blockage creates pressure on the inside
of the intestine. This causes part of the intestinal wall to wear thin. Over time, a
bacterial infection called enterocolitis can develop in the digestive tract. This is a
serious complication involving the following signs and symptoms:
1. Fever
2. Swollen belly
3. Vomiting
4. Diarrhea
5. Bleeding from the rectum
6. Fatigue. (Stanford Children’s Health)
II. MEDICAL INTERVENTIONS & TREATMENT
a. DIAGNOSTIC EVALUATION
i. Diagnosis is often via barium enema and rectal biopsy, since the disease is often
only limited to the colon. A barium enema is an x-ray exam that detects changes
or abnormalities in the large intestine (colon). An enema is the injection of a
liquid into the rectum through a small tube. A rectal biopsy on the other hand, is
a procedure used to extract a tissue sample from the rectum for laboratory
analysis.
ii. Occasionally, rectal manometry is performed; this is a test used to measure and
assess pressure, reflex and sensation in the rectum, as well as evaluate the
efficiency of the anal sphincter.
iii. Other diagnostic exams may be ordered by the physician such as laboratory
studies and plain abdominal radiography.
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b. MEDICAL MANAGEMENT
i. Treatment for mild to moderate disease is based on relieving the chronic
constipation with stool softeners and rectal irrigations; however, many children
require surgery.
ii. 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.
iii. Decompression. Decompression can be accomplished through placement of a
nasogastric tube and either digital rectal examination or normal saline rectal
irrigations3-4 times daily.
iv. Diet. A special diet is not required; however, preoperatively and in the early
postoperative period, infants on a non-constipated regimen, such as breast milk,
are more easily managed.
v. Pharmacologic Management
1. Drug therapy is not a component of the standard of care for this disease
itself; however, some medications may be used to treat its
complications.
2. Antibiotics. Administer broad-spectrum antibiotics to patients with
enterocolitis.
c. SURGICAL TREATMENT
i. Treatment for moderate to severe disease involves a 2-step surgical procedure.
1. Leveling colostomy. Initially, in the neonatal period, a temporary
colostomy is created to relieve obstruction and allow the normally
innervated, dilated bowel to return to normal size.
2. Single-stage pull-through procedure. When the bowel returns to its
normal size, a complete surgical repair is performed via a pull through
procedure to excise portions of the bowel; at this time, the colostomy is
closed.
III. NURSING INTERVENTIONS
a. Nursing Interventions: General
i. Assessment involves:
1. History. Carefully gather a history from the family caregivers, noting
especially the history of stooling; ask about the onset of constipation,
the character and odor of stools, the frequency of bowel movements,
and the presence of poor feeding habits, anorexia, and irritability.
2. Physical Examination. During the physical exam, observe for distended
abdomen and signs of poor nutrition; record weight and vital signs.
ii. Promote skin integrity. When performing routine 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
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with skin-toughening preparations that strengthen it and provide better

adhesion of the appliance.
iii. Promote comfort. Observe for signs of pain, such as crying, pulse and
respiration rate increases, restlessness, guarding of the abdomen, or drawing up
the legs; administer analgesics as ordered; additional nursing measures that can
be used are changing the child’s position, holding the child when possible,
stroking, cuddling, and engaging in age-appropriate activities.
iv. Maintain fluid balance. Accurate intake and output determinations and
reporting the character, amount, and consistency of stools help determine
when the child may have oral feedings; to monitor fluid loss, record and report
the drainage from the NG tube every 8 hours; and immediately report any
unusual drainage, such as bright-red bleeding.
v. Provide oral and nasal care. Perform good mouth care at least every 4 hours; at
the same time, gently clean the nares to relieve any irritation from the NG tube.
vi. Provide family teaching. Show the family caregiver how to care for the
colostomy at home; discuss topics such as devices and their use, daily irrigation,
and skin care; the caregivers should demonstrate their understanding by caring
for the colostomy under the supervision of nursing personnel several days
before discharge.
b. Interventions: Medical Management
i. Maintain a low-fiber, high-calorie, high-protein diet; parenteral nutrition may be
necessary in extreme situations.
ii. Administer stool softeners as prescribed.
iii. Administer daily rectal irrigations with normal saline to promote adequate
elimination and prevent obstruction as prescribed.
c. Pre-operative Interventions: Surgical Management
i. Assess bowel function.
ii. Administer bowel preparation as prescribed.
iii. Maintain NPO status.
iv. Monitor hydration and fluid and electrolyte status; provide fluids intravenously
as prescribed for hydration.
v. Administer antibiotics or colonic irrigations with an antibiotic solution as
prescribed to clear the bowel of bacteria.
vi. Monitor strict intake and output.
vii. Obtain daily weight.
viii. Measure abdominal girth daily.
ix. Avoid taking the temperature rectally.
x. Monitor for respiratory distress associated with abdominal distention.
d. Post-operative Interventions: Surgical Management
i. Monitor vital signs, avoiding taking the temperature rectally.
ii. Measure abdominal girth daily and PRN (as needed).
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iii. Assess the surgical site for redness, swelling and drainage.
iv. Assess the stoma if present for bleeding or skin breakdown (stoma should be
red and moist).
v. Assess the anal area for the presence of stool, redness or discharge.
vi. Maintain NPO status as prescribed and until bowel sounds return or flatus is
passed, usually within 48 to 72 hours.
vii. Maintain nasogastric tube to allow intermittent suction until peristalsis returns.
viii. Maintain IV fluids until the child tolerates appropriate oral intake, advancing the
diet from clear liquids to regular as tolerated and as prescribed.
ix. Assess for dehydration and fluid overload. Monitor strict intake and output and
obtain daily weight.
x. Assess for pain and provide comfort measures as required.
xi. Provide the parents with instructions regarding colostomy care and skin care.
xii. Teach the parents about the appropriate diet and the need for adequate fluid
intake.
IV. NURSING CARE PLAN (2)
Patient R arrives at the Emergency Room accompanied by his mother/ caretaker:
 3 years old, Male.

 History of 2 weeks of absent bowel movement,
 chronic constipation,
 foul-smelling ribbon-shaped stool,
 nausea and vomiting (2-3 times per day for the past week)
 abdominal distention,
 Underweight, BMI= 14,
 Fatigue & lack of appetite
Assessment Nursing Planning Interventions Rationale Evaluation

Diagnosis
Subjective: Altered Bowel After 8 hours 1. Assess the 1. Identify the After 8 hours
Elimination of nursing patient’s baseline data of nursing
“Mahigit
related to interventions, pattern of for elimination interventions,
dalawang
hypertrophy the patient and elimination, pattern and the patient and
linggo na hindi
and distention corresponding stool color, character of corresponding
dumudumi ang
of proximal caretaker will consistency stool, if any. caretaker were
anak ko.” (My
colon. demonstrate frequency and able to
baby has not
changes in amount. demonstrate
pooped for
behavior as 2. Adequate changes in
more than a 2. Encourage
necessitated fluid intake can behavior as
week.) mother of
by causative improve the necessitated
patient to
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“Para pating and administer balance by causative

nasakit ang contributing water intake of between and
tiyan niya, lagi factors. 1500 to 2000 absorption in contributing
siya naiyak at cc/day to the colon and factors.
hindi patient. fluid intake,
nakakatulog.” thereby
(And it seems preventing the
as if his tummy formation of
hurts. He hard feces –
keeps crying promoting
and can’t easier passage
sleep) as of stool.
verbalized by 3. Recommend
avoiding gas- 3. Decreases
the patient’s
forming foods. gastric distress
mother.
and abdominal
distention.
Objective: 4. Assess peri- 4. Prevents

anal skin skin
(+) abdominal condition excoriation
distention frequently, and
(+) hyperactive noting changes breakdown.
bowel sounds or beginning of
in the breakdown.
epigastric and Encourage or
right lower and assist with
upper perianal care
quadrants; after each
bowel
(-) bowel movement.
sounds in the
left lower 5. Discuss use 5. Facilitates
quadrant of stool defecation
softeners, mild when
Patient stimulants, constipation is
underweight, enemas as present.
(+) anorexic indicated by
appearance treatment
BMI= 14 regimen.
Monitor
effectiveness
of
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interventions.
Collaborative:
6. Consult with 6. Fiber resists

dietician to enzymatic
provide well- digestion and
balanced diet absorbs liquids
high in fiber in its passage
and bulk. along the
intestinal tract
and thereby
produces bulk,
which acts as a
stimulant for
defecation.

Diagnosis
Subjective: Imbalanced After 8 hours 1. Monitor 1. Adequate

Nutrition: Less of nursing food intake. intake of food
“Wala na gana
than Body interventions, contributes to
kumain anak
Requirements the patient the healing
ko…dalwa
related to and/or process.
hanggang tatlong
nausea, corresponding
beses yan 2. Provision of 2. Eating small,
vomiting, and caretaker will:
magsuka sa isang adequate frequent
mal-
araw. Payat na > present calories and meals lessens
absorption as
payat na.” (My understanding balanced the feeling of
evidenced by
son has no of significance meals via fullness and
less than
appetite…he of nutrition to small, decreases the
normal body
vomits 2-3 times healing nutrient- stimulus to
weight
in a day. He’s so process and dense vomit.
thin now.) as general health frequent
verbalized by the feedings (5-6
> decreased
patient’s times per day,
episodes of
caretaker/mother. ice chips if
nausea and
patient is too
Objective vomiting
nauseated to
PR: 150 bpm >increased eat).
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RR: 45 bpm energy levels 3. Vomiting

decreases the
(+) lethargic
amount of
(+) cold sweats 3. Monitor fluid and
hydration and nutrients
(+) abdominal absorbed in
fluid and
pain, evidenced the body.
electrolyte
by facial grimace,
status;
guarding of
provide fluids
abdominal area,
intravenously 4. Elevating
and near-constant
as prescribed the head of
crying.
for hydration. bed 30
Patient degrees aids in
4. Promote
underweight, (+) swallowing
proper
anorexic and reduces
positioning.
appearance BMI= risk for
14 aspiration with
eating.
5. Vomitus is
often acidic
and can
damage the
5. Provide oral cavity
good oral especially the
hygiene and teeth if too
frequent frequent.
saline washes. Providing oral
hygiene and
saline washes
can help
prevent this.
6. Nutritional
6. Consider
support may
the possible
be
need for
recommended
parenteral
for patients
nutritional
who are
support with
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the patient, unable to

family and maintain
caregiver as nutritional
appropriate. intake by the
oral route.
**In the case of:
Neonatal patient until pre-toddler stage diagnosed with

Hirschsprung’s Disease with Colostomy:
Pre-colostomy neonatal appearance, notable abdominal

distention secondary to intestinal obstruction caused by
Hirschsprung’s Disease:
Image taken from

omicsonline.org.
Post-colostomy procedure, resolved abdominal distention/ intestinal obstruction:

Lucena City
Sample NCP for Hirschsprung Disease patient with colostomy

Diagnosis
Subjective: Risk for Short Term: 1. Assess skin, 1. Establish Short Term:
impaired skin note color, comparative
-------------- After 4 hours After 4 hours of
integrity turgor, baseline data
of nursing nursing
Objective: related to sensation; providing
interventions, interventions,
irritation from describe and opportunity
Presence of the patient will the
the colostomy measure for efficient
stoma in the participate in patient/caretaker
stoma and and timely
right lower prevention shall participate
observe for interventions.
quadrant of measures and in prevention
changes.
the abdomen treatment measures and
regimen 2. Instruct treatment
The patient 2. Skin friction
accordingly. family regimen
may manifest: caused by stiff
caretaker to accordingly.
or rough
>Pain, maintain
Long Term: clothes leads Long Term:
itchiness, patient in
to irritation
swelling of the After 3 days of clean and dry, After 3 days of
and increases
skin around nursing preferably nursing
risk for
the stoma interventions, cotton fabric interventions,
infection.
the patient/ clothes. the patient/
>infection caretaker will caretaker shall
3. Instruct the
demonstrate demonstrate
patient/ 3. To provide
increased self- increased self-
caretaker that proper ostomy
esteem and esteem and
the peristomal care and
comfortable comfortable
area should be prevent
adjustment adjustment with
cleaned well complications.
with changing changing stoma
with a mild
stoma pouch pouch
soap and dried
independently independently
before the
and patient and patient will
new pouch is
will display display timely
applied.
timely wound wound healing.
healing. 4. Instruct the
Evidenced by
patient / 4. To increase
Also evidenced absence of
caretaker that the caretaker’s
by absence of rashes and skin
the pouch knowledge on
rashes and irritations around
should be proper ostomy
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skin irritations changed every care. the stoma and

around the 4-5 days or caretaker was
stoma and was when leakage able to identify
able to identify occurs individual factors
individual that may
5. Teach the 5. The
factors that contribute to
patient’s patient’s
may skin breakdown.
caretaker to caretaker
contribute to
empty the should
skin
pouch when it demonstrate
breakdown.
is about half the ability to
full and teach empty and
them how to change the
clean out the pouch
pouch independently.
properly
without
emptying it.
6. Discuss the 6. These health

importance of teachings
adequate provide the
nutrition caretaker with
especially information on
fluids, protein, how nutrition
vitamin C, can elevate the
vitamin B, patient’s
iron, calories chances of
and faster
potassium-rich recovery.
foods.
7. This is
7. Instruct the necessary to
caretaker in gather more
stoma data
assessment concerning the
and provide patient’s
mechanism for condition thus,
documenting. identifying skin
problems and
promoting
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comfortable
adjustment to
the colostomy.
8. Instruct to
8. Protects the
apply skin
skin from
barrier (ie.
adhesive and
karaya
waste enzyme
powder) to the
irritation.
surrounding
area.
Dependent:
9. Apply 9. Assists in
antifungal healing if
powder as irritation
prescribed by persists.
physician.
USED SOURCES LINKS:
https://nursingcrib.com/nursing-notes-reviewer/hirschsprungs-disease/
https://www.msdmanuals.com/professional/pediatrics/congenital-gastrointestinal-
anomalies/hirschsprung-disease
https://emedicine.medscape.com/article/929733-overview
https://www.stanfordchildrens.org/en/topic/default?id=hirschsprungs-disease-90-P01999#
https://health.ucdavis.edu/internalmedicine/gastro/rectalmanometry.html
https://www.mayoclinic.org/tests-procedures/barium-enema/about/pac-20393008
https://www.healthline.com/health/rectal-biopsy
https://www.omicsonline.org/israel/hirschsprungs-disease-peer-reviewed-pdf-ppt-articles/

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Manuel S.

Enverga University Foundation

COLLEGE OF NURSING ALLIED HEALTH SCIENCES

In partial fulfillment of the requirements for

NURSING CASE STUDY

I. BACKGROUND OF THE DISEASE

Image above, pediatric hirschsprung

The etiology of aganglionosis is thought to be

1. Having a sibling who has Hirschsprung’s disease. Hirschsprung’s

d. SIGNS AND SYMPTOMS

with skin-toughening preparations that strengthen it and provide better

Patient R arrives at the Emergency Room accompanied by his mother/ caretaker:

 3 years old, Male.

Assessment Nursing Planning Interventions Rationale Evaluation

“Para pating and administer balance by causative

Objective: 4. Assess peri- 4. Prevents

6. Consult with 6. Fiber resists

Assessment Nursing Planning Interventions Rationale Evaluation

Subjective: Imbalanced After 8 hours 1. Monitor 1. Adequate

RR: 45 bpm energy levels 3. Vomiting

the patient, unable to

**In the case of:

Neonatal patient until pre-toddler stage diagnosed with

Pre-colostomy neonatal appearance, notable abdominal

Image taken from

Post-colostomy procedure, resolved abdominal distention/ intestinal obstruction:

Sample NCP for Hirschsprung Disease patient with colostomy

Assessment Nursing Planning Interventions Rationale Evaluation

skin irritations changed every care. the stoma and

6. Discuss the 6. These health

USED SOURCES LINKS:

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