I.

Learning Objectives:
- This case study of Hirschsprung's disease will give me somehow knowledge about the disease and eventually will
give me ideas on how to give a quality nursing care on how to manage its symptoms if ever I will encounter a
disease like this in the near future.
II. INTRODUCTTION
Definition
Hirschsprung's disease, also known as congenital megacolon or aganglionic megacolon, is an abnormality in which
certain nerve fibers are absent in segments of the bowel, resulting in severe bowel obstruction. It was first
identified in 1886 by a physician named Harold Hirschsprung.
Hirschsprungs disease (Congenital Aganglionic Megacolon) is the congenital absence of or arrested development
of parasympathetic ganglion cells in the intestinal wall, usually in the distal colon. Signs and symptoms may vary
with the severity of the condition. Sometimes they appear right after the baby is born. Other times they may not
be apparent until the baby becomes a teenager or adult.
In newborns, signs may include:
-Failure to pass stool within the first or second day of life
-Vomiting, including vomiting a green liquid called bile a digestive fluid produced in the liver
-Constipation or gas, which may make a newborn fussy
-Diarrhea

In older children, signs can include:
-Swollen abdomen
-Lack of weight gain
-Problems absorbing nutrients, leading to weight loss, diarrhea or both and delayed or slowed growth
-Infections in the colon, especially in newborns or very young children, that may include enterocolitis, a
serious infection with diarrhea, fever and vomiting and sometimes a dangerous expanding (dilation) of
the colon
-In older children or adults, signs may include chronic constipation and a low number of red blood
cells (anemia) because blood is lost in the stool.

Description
Hirschsprung* disease (HD) is a disease of the large intestine that causes severe constipation or intestinal
obstruction. Constipation means stool moves through the intestines slower than usual. Bowel movements occur
less often than normal and stools are difficult to pass. Some children with Hirschsprung disease can't pass stool at
all, which can result in the complete blockage of the intestines, a condition called intestinal obstruction. People
with Hirschsprung disease are born with it and are usually diagnosed when they are infants. Less severe cases are
sometimes diagnosed when a child is older. An Hirschsprung disease diagnosis in an adult is rare.
Hirschprung's disease occurs once in every 5,000 live births, and it is about four times more common in males than
females. Between 4% and 50% of siblings are also afflicted. The wide range for recurrence is due to the fact that
the recurrence risk depends on the gender of the affected individual in the family (i.e., if a female is affected, the
recurrence risk is higher) and the length of the aganglionic segment of the colon (i.e., the longer the segment that
is affected, the higher the recurrence risk).
Why does Hirschsprung's disease cause constipation?
People with Hirschsprung disease have constipation because they lack nerve cells in a part or all of the large
intestine. The nerve cells signal muscles in the large intestine to push stool toward the anus. Without a signal to
push stool along, stool will remain in the large intestine.
In a healthy large intestine the nerve cells are found throughout the large intestine.
Short-segment Hirschsprung disease. Nerve cells are missing from the last segment of the large intestine.
Long-segment Hirschsprung disease. Nerve cells are missing from most or all of the large intestine and sometimes
the last part of the small intestine.
How severe Hirschsprung disease is depends on how much of the large intestine is affected. Short-segment
Hirschsprung disease means only the last part of the large intestine lacks nerve cells. Long-segment Hirschsprung
disease means most or all of the large intestine, and sometimes the last part of the small intestine, lacks nerve
cells.
In a person with Hirschsprung disease, stool moves through the large intestine until it reaches the part lacking
nerve cells. At that point, the stool moves slowly or stops, causing an intestinal obstruction.
Causes and symptoms
Before birth, a child's nerve cells normally grow along the intestines in the direction of the anus. With Hirschsprung
disease, the nerve cells stop growing too soon. Why the nerve cells stop growing is unclear. Some Hirschsprung
disease is inherited, meaning it is passed from parent to child through genes. Hirschsprung disease is not caused by
anything a mother did while pregnant.
The initial symptom is usually severe, continuous constipation. A newborn may fail to pass meconium (the first
stool) within 24 hours of birth, may repeatedly vomit yellow- or green-colored bile and may have a distended
(swollen, uncomfortable) abdomen. Occasionally, infants may have only mild or intermittent constipation, often
with diarrhea.
While two-thirds of cases are diagnosed in the first three months of life, Hirschsprung's disease may also be
diagnosed later in infancy or childhood. Occasionally, even adults are diagnosed with a variation of the disease. In
older infants, symptoms and signs may include anorexia (lack of appetite or inability to eat), lack of the urge to
move the bowels or empty the rectum on physical examination, distended abdomen, and a mass in the colon that
can be felt by the physician during examination. It should be suspected in older children with abnormal bowel
habits, especially a history of constipation dating back to infancy and ribbon-like stools.
Diagnosis
Hirschsprung's disease in the newborn must be distinguished from other causes of intestinal obstruction. The
diagnosis is suspected by the child's medical history and physical examination, especially the rectal exam. The
diagnosis is confirmed by a barium enema x ray, which shows a picture of the bowel. The x ray will indicate if a
segment of bowel is constricted, causing dilation and obstruction. A biopsy of rectal tissue will reveal the absence
of the nerve fibers. Adults may also undergo manometry, a balloon study (device used to enlarge the anus for the
procedure) of internal anal sphincter pressure and relaxation.
Patients Profile
Name: Candera, Jaybie
Sex: Female
Date Admitted: March 27, 2014
Date of Birth: December 23, 2013
Address: Brgy. San. Vicente Sta. Maria Bulacan
Chief Complaint: Abdominal Enlargement
Medical Diagnosis: Hirshprungs Disease

III. ANATOMY AND PHYSIOLOGY
The patient with a complaint related to the pelvic floor (e.g. incontinence, constipation, prolapse, or obstructed
defecation) can be studied with a variety of tests to help determine the etiology of the disorder and direct therapy.
Anorectal physiology testing, endoanal ultrasound, and defecography are used to evaluate patients prior to
surgery (e.g. anal sphincter defects, rectal prolapse), help delineate difficult anatomy (e.g. recurrent anal fistulas,
rectovaginal fistulas), and to determine if a non-surgical approach or biofeedback is appropriate (nonrelaxing
puborectalis, incontinence). An understanding of the anatomy and physiology of the pelvic floor is necessary to
both choose the appropriate tests and interpret the results.



Large intestine, colon, rectum, and anus
The large intestine, which includes the colon and rectum, is the last part of the digestive tract. The large intestine's
main job is to absorb water and hold stool. The rectum connects the colon to the anus. Stool passes out of the
body through the anus. At birth, the large intestine is about 2 feet long. An adult's large intestine is about 5 feet
long.

Pelvic Floor Muscles
The pelvic floor consists of a striated muscular sheet through which viscera pass. This striated muscle, the paired
levator ani muscles, is actually subdivided into four muscles defined by the area of attachment on the pubic bone.
The attachments span from the pubic bone, along the arcus tendineus (a condensation of the obturator fascia), to
the ischial spine. The components of the levator ani are therefore named the pubococcygeus, ileococcygeus, and
ischiococcygeus. The pubococcygeus is further subdivided to include the puborectalis. Between the urogenital
viscera and the anal canal lies the perineal body. The perineal body consists of the superficial and deep transverse
perinei muscles and the ventral extension of the external sphincter muscle to a tendinous intersection with the
bulbocavernosus muscle. The postanal plate lies between the anus and the veterbral column and consists of the
presacral fascia, the anococcygeal ligament, anococcygeal raphe (midline condensation of the ileococcygeus), and
the dorsal extension of the puborectalis and external anal sphincter fibers to the coccyx.
Pelvic Floor Innervation
The fourth sacral nerve innervates the levator ani muscles. Controversy continues regarding the innervation and
origin of the puborectalis muscle. Cadaver studies differ from in vivo stimulation studies as to whether the
puborectalis muscle receives innervation only from the sacral nerve or also from the pudendal nerve. Comparative
anatomy and histological studies of fiber typing also support the inclusion of the puborectalis muscle with the
sphincter complex and not as a pelvic floor muscle. In addition, EMG studies of the external anal sphincter and
puborectalis muscle indicate that the muscles function together with cough and strain.
Rectum and Anus
The anal canal is approximately 4 cm long and extends from the anal verge to the top of the external anal sphincter
complex or ring. This is a clinical and surgical description that corresponds to the digital exam and findings seen on
ultrasound but not to the histological changes along the canal. It differs from a purely anatomical description of
the anal canal as extending from the anal verge to the dentate line or a histological one which describes the anal
canal as ending at approximately 10 mm above the anal valves where it is lined by rectal type mucosa. The dentate
line lies at or just distal to the anal valves. It is a mucosal boundary and not the proximal boundary of the
transitional zone. The anal transitional zone, as defined by Fenger, is the "zone interposed between uninterrupted
colorectal type mucosa above and uninterrupted squamous epithelium below."
Rectal Muscles
The rectal smooth muscle consists of the muscularis mucosa, and inner circular layer and an outer longitudinal
layer. The inner circular muscle forms the valves of Houston and in the proximal 30 mm of the 40-mm anal canal it
becomes the internal anal sphincter. Ultrasound examination of the anal canal clearly shows that the internal anal
sphincter ends approximately 10 mm proximal to the most distal portion of the external anal sphincter. The outer
longitudinal layer surrounds the sigmoid colon but is thicker the areas known as the taenia coli. This same layer
continues down to the anorectal junction where it forms the conjoined longitudinal muscle along with fibers from
the pubococcygeus muscle. Distally, this muscle lies in the intersphincteric plane and fibers may fan out and cross
both the internal and external anal sphincter muscles.
Internal Anal Sphincter
The internal anal sphincter is an involuntary, smooth muscle. It receives sympathetic innervation via the
hypogastric and pelvic plexus. Parasympathetic innervation is from S1, S2, and S3 via the pelvic plexus. There is
considerable evidence that the sympathetic innervation is excitatory but conflicting information regarding the
parasympathetic effect. There is an unclear contribution to the innervation from nonadrenergic, noncholinergic
pelvic nerves. Spinal anesthesia decreases rectal tone by 50% and the decreased resting tone seen in diabetic
patients may be due to an autonomic neuropathy. The internal anal sphincter contributes 55% to the anal resting
pressure. The myogenic activity contributes 10% and 45% is due to the sympathetic innervation. The remainder of
the resting tone is from the hemorrhoidal plexus (15%) and the external anal sphincter (30%). The internal anal
sphincter has slow waves occurring 6-20 times each minute. Ultraslow waves occur less than 3 times a minute and
are not present in all individuals. Ultraslow waves are associated with higher resting pressures, hemorrhoids and
anal fissures.
External anal sphincter
The external anal sphincter was classically described as having three components (subcutaneous, superficial, and
deep), but is now regarded as one continuous muscle. The proximal aspect is in intimate relationship with the
puborectalis muscle. The pudendal nerve, specifically the inferior rectal branch, innervates the external anal
sphincter.
Rectal and anal sensory innervation
Anal canal sensation to touch, pin-prick, heat, and cold are present from the anal verge to 2.5 - 15 mm above the
anal valves. This sensitive area is thought to help discriminate between flatus and stool but local anesthesia does
not obliterate that ability. The rectum is only sensitive to distention. Rectal sensation may be due to receptors in
the rectal wall but also in the pelvic fascia or surrounding muscle. The sensory pathway for rectal distention is the
parasympathetic system via the pelvic plexus to S2, S3 and S4. Below 15 cm rectal distention is perceived as flatus
but above 15 cm air distention causes a sensation of abdominal discomfort. Anal canal sensation is via the inferior
rectal branch of the pudendal nerve that arises from S2, S3 and S4. This is the first branch of the pudendal nerve
and along with the second branch, the perineal nerve, arises from the pudendal nerve in the pudendal canal
(Alcocks canal). The remainder of the pudendal nerve continues as the dorsal nerve of the penis or clitoris.

Vascular supply of the rectum and anus
The superior and inferior rectal arteries supply the rectum and anal canal. The superior rectal artery is a
continuation of the inferior mesenteric artery as it passes over the left common iliac artery. The inferior rectal
artery is a branch of the pudendal artery, which arises from the internal iliac artery. The middle rectal artery is a
branch of the anterior division of the internal iliac artery Controversy is associated with the contribution of the
middle rectal artery. Conflicting cadaver studies suggest that it is present 5 - 70% unilaterally or bilaterally. The size
and presence of the middle rectal artery may be in inverse relationship to the superior rectal artery. As a practical
matter, a particularly small superior rectal artery may be an indication during a low anterior resection that the
middle rectal arteries are substantial. Less controversial is the paucity of vascular supply to the anterior and
particularly posterior aspects of the rectum from the superior rectal artery. The extent of crossover of vascular
supply between the inferior and superior rectal arteries is also debated however it is accepted that the rectum and
anus can survive ligation of the superior and middle rectal arteries. Venous drainage follows the main arterial
supply.
Reflexes
Rectoanal inhibitory reflex and Inflation reflex
Rectal distention and electrical stimuli induce relaxation of the internal anal sphincter with a resultant lowering of
the resting pressure. The internal anal sphincter recovers within one minute from small volumes of air or stool. . A
reflex contraction of the external anal sphincter (inflation reflex or rectoanal excitatory reflex) is seen at the time
of the internal anal sphincter relaxation except during sleep. This inflation reflex is lost at high rectal volumes (400
ml). The rectoanal inhibitory reflex is absent in Hirschsprung's disease, Chagas disease, and after rectal mucosal
excision. It may return after initially being abolished by a low rectal anastomosis. The afferent receptors are
therefore not only in the anorectal epithelium and rectal wall, but probably also in the pelvic floor. The reflex is
intramural involving myenteric plexus neurons that are noncholinergic and nonadrenergic.
Sampling Reflex
The sampling reflex is incompletely understood but a vital component of continence. Stool or flatus entering into
the rectum causes a reflex relaxation of the internal anal sphincter. The length of the functional high-pressure zone
of the anal canal decreases and this allows contact of the rectal contents with the highly sensitive anal canal. A
deficit in this sensory function can be a contributing factor in patients with fecal incontinence in the absence of
motor deficits. Biofeedback can improve rectal balloon distention thresholds.
Anal Cough Reflex
The external anal sphincter contracts with coughing, sneezing, and standing. The squeeze pressure with cough is
higher than when the patient voluntarily contracts the external anal sphincter muscle.
Cutaneoanal reflex
The cutaneoanal reflex or "anal wink" has its afferent and efferent pathways in the pudendal nerve and is
abolished if S4 is transected. The latency can vary widely with short, intermediate and long latencies. Short
latencies are thought to be due to direct stimulation of nerve fibers near the sphincter. Intermediate latencies are
probably due to stimulation to a branch point that then travels back to the muscle failing to complete the
complete reflex arc at the spinal level. This variability in latency limits usefulness of measurement of this reflex.


Defecation
The awareness of the need to defecate or urinate occurs in the superior frontal gyrus and anterior cingulate gyrus.
Rectal distention stimulates internal anal sphincter relaxation and the sampling reflex. If defecation is to be
deferred voluntary contraction of the external anal sphincter and levator ani muscles occurs. Accommodation
refers to the relaxation of the rectal ampulla after an initial increase in pressure. At the appropriate time for
defecation or when rectal pressure is high, the levator ani muscle, puborectalis muscle and external anal sphincter
relax. Pelvic floor relaxation, along with a squatting position, straightens the anorectal angle. An increase in
abdominal pressure along with colonic and rectal contractions allows expulsion of a fecal bolus. Increased effort is
necessary to expel a smaller bolus than an optimal 2-cm fecal bolus.
Continence
The interplay of all the aforementioned anatomy and physiology ensures continence. It does not follow that a
deficit in any one area ensures incontinence. Continence achieved in the ileoanal pouch patient is proof the rectum
is not essential. An intact and functional puborectalis muscle can provide continence in the pediatric imperforate
anus patient, but incontinence can ensue during adulthood. Even profound deficits do not necessarily lead to
incontinence if stool consistency is solid, while minor deficits can easily lead to incontinence to gas and a
percentage of normal adults have gas incontinence. This complex problem requires a systematic approach focusing
on identifying the specific deficits present and then directing therapy accordingly.
IV. PATHOPHYSIOLOGY
Male>Female Diet, Activity, Race

Absence of Ganglion cells

(-) ganglionic innervation = (-) nerve cell

Chronic constipation (-) Peristalsis

Intestinal Obstruction / discomfort Imapaction Red, Dark, Blood Stool
Anemia
Shock
Abdominal Fullness / Distention ABDOMINAL PAIN

DEATH






VI.DRUG STUDY:
DRUGS NAME CLASSIFICATION INDICATION CONTRAINDICATIO
N
SIDE EFFECT NURSING
CONSIDERATION

Ampicillin
Sodium

Antibiotic
Penicillin
Ampicillin is
an antibiotic
in the
penicillin
group of
drugs. It
fights
bacteria in
your body.
Ampicillin is
used to
treat many
different
types of
infections
caused by
bacteria,
such as ear
infections,
bladder
infections,
pneumonia,
gonorrhea,
and E. coli
or
salmonella
infection.


Contraindicated
with allergies to
penicillins,
cephalosporins,
or other allergens
Use cautiously
with renal
disorder.


*Nausea,
vomiting, diarrhea, or
mouth/tongue sores
may occur. If any of
these effects persist
or worsen, notify
your doctor or
pharmacist promptly
*Ampicillin can
commonly cause a
mild rash that is
usually not serious.
However, you may
not be able to tell it
apart from a rare
rash that could be a
sign of a severe
allergic reaction.
Therefore, seek
immediate medical
attention if you
develop any rash.
Do not use this
medication if you
are allergic to
ampicillin or to
any other
penicillin
antibiotic, such as
amoxicillin.
Before using
ampicillin, tell
your doctor if you
are allergic to
cephalosporins
such as Ceclor,
Ceftin, Duricef,
Keflex, and
others, or if you
have asthma,
kidney disease, a
bleeding or blood
clotting disorder,
mononucleosis
(also called
"mono"), or a
history of any
type of allergy..
Take this
medication for
the full prescribed
length of time.
Your symptoms
may improve
before the
infection is
completely
cleared.




DRUGS NAME CLASSIFICATION INDICATION CONTRAINDICATION SIDE EFFECTS NURSING RESPONSIBILITY

Gentamicin
Sulfate


antiinfective;
aminoglycosie
antibiotic


Parenteral use
restricted to
treatment of
serious infections
of GI, respiratory,
and urinary tracts,
CNS, bone, skin,
and soft tissue
(including burns)
when other less
toxic
antimicrobial
agents are
ineffective or are
contraindicated.
Has been used in
combination with
other antibiotics.
Also used
topically for
primary and
secondary skin
infections and for
superficial
infections of
external eye and
its adnexa.


History of
hypersensitivity
to or toxic
reaction with any
aminoglycosidean
tibiotic. Safe use
during pregnancy
(category C) or
lactation is not
established

Hypersensitivity
(rash, pruritus,
urticaria,
exfoliative
dermatitis,
eosinophilia,
burning sensation
of skin, drug fever,
joint pains,
laryngeal edema,
anaphylaxis).
Urogenital:
Nephrotoxicity:
proteinuria,
tubular necrosis,
cells or casts in
urine, hematuria,
rising BUN,
nonprotein
nitrogen, serum
creatinine;decreas
ed creatinine
clearance. Other:
Local irritation and
pain following IM
use;
thrombophlebitis,
abscess,
superinfections,
syndrome of
hypocalcemia
(tetany, weakness,
hypokalemia,
hypomagnesemia).

Assessment & Drug Effects
Lab tests: Perform C&S and
renal function prior to first
dose and periodically during
therapy; therapy may begin
pending test results.
Determine creatinine
clearance and serum drug
concentrations at frequent
intervals, particularly for
patients with impaired renal
function, infants (renal
immaturity), older adults,
patients receiving high
doses or therapy beyond 10
d, patients with fever or
extensive burns, edema,
obesity.
Repeat C&S if improvement
does not occur in 35 d;
reevaluate therapy.
Note: Dosages are generally
adjusted to maintain peak
serum gentamicin
concentrations of 4 10
g/mL, and trough
concentrations of 12 g/mL.
Peak concentrations above
12 g/mL and trough
concentrations above 2
g/mL are associated with
toxicity.
Draw blood specimens for
peak serum gentamicin
concentration 30 min1h
after IM administration, and
30 min after completion of
a 3060 min IV infusion.
Draw blood specimens for
trough levels just before the
next IM or IV dose. Use
nonheparinized tubes to
collect blood.


VII. NURSING CARE PLANS:
ASSESSMENT DIAGNOSIS INFERENCE PLANNING INTERVENTION RATIONALE EVALUATION

Subjective:

Hindi madumi
ang anak ko,
lumalaki ang
kanyang tiyan
simula nung
lunes po

Objective:

Altered bowel
sounds.
Abdominal
enlargement
Reports of
abdominal pain
or cramping.
V/S taken as
follows:

T: 37.5
P: 130
R: 30


Constipation
related to
changes
indigestive
process.

Hirschsprungsdisea
se(congenitalaganglionic
megacolon) is the
congenital absence of or
arrested development
of parasympathetic
ganglion cells in the
intestinal wall, usually in
the distal colon.
Symptoms are related
to chronic intestinal
obstruction and usually
appear shortly after
birth but may not be
recognized until
later in childhood
or(rarely) in
adulthood. In this order
the lack of colorectal
innervation
inhibits peristalsis,
and the affected
portion of intestine
become spastic and
contracted. The internal
rectal sphincter fails
to relay, which prevents
evacuation of fecal
material and gas and
causes severe
abdominal distention
and constipation

After 8 hours of
nursing
interventions, the
patient will
demonstrate
changes in
behavior as
necessitated by
causative and
contributing
factors
Independent:

Determine stool
color, consistency,
frequency, and amount.



Auscultate bowel
sounds.

Monitor intake and
output (I&O)with
specific attention to
food or fluid intake.

Recommend
avoiding gas forming
foods.

Assess perianal skin
condition frequently,
noting changes or
beginning breakdown.
Encourage or assist
with perineal care
after each bowel
movement.
Discuss use of stool
softeners, mild
stimulants, enemas as
indicated. Monitor
effectiveness
Collaborative:
Consult with
dietitian to provide
well balanced diet high
in fiber and bulk.



Assists in
identifying
causative or
contributing factors
and appropriate
intervention.
Bowel sounds are
generally increased in
constipation.

May identify
dehydration,
excessive loss of
fluids or aid in
identifying dietary
deficiencies.
Decreases gastric
distress and
abdominal
distension.
Prevents skin
excoriation and
breakdown.






Facilitates
defecation when
constipated is
present.


Fiber resist
enzymatic digestion
and absorbs liquids in
its passage along the
intestinal tract and
thereby produces
bulk, which acts as a
stimulant for
defecation.
After 8 hours of
nursing
interventions, the
patient was able
to demonstrate
changes in
behavior as
necessitated by
causative and
contributing
factors


ASSESSMENT DIAGNOSIS INFERENCE PLANNING INTERVENTION RATIONALE EVALUATION

Subjective:
kanina pa siya
iyak ng iyak
hindi ko na
alam ang
gagawin ko.

Objective:
-crying
-irritable
-wrinkling
eyebrow
-facial grimace
-change in
respiratory
and heart rate
-hyperactive
arms and legs

Alteration in
Comfort: Pain
related to
distended
colon
secondary to
lack of
innervation in
the colon.

A common
symptom of a
distended colon
is abdominal
pain. As the
colon enlarges,
either because
of an
obstruction or pse
udo-
obstruction, the
stretching of the
colon can cause
pain and
cramping. The
abdomen c
also become
visibly distended,
resulting in a
large, protruding
abdomen. The
abdomen may
also become
very tender to
touch.

After several
hours of
nursing
intervention
s, the
patient will
be able to
demonstrat
e relief of
pain from
pain scale
5/5 to pain
scale 2/5
(Wong-
Baker faces
Pain Rating
Scale


Independent:

Assessed pain
using facial
expression scale
that is appropriate
to the age.
Observed
nonverbal cues of
pain and other
objective defining
characteristic.
Provided comfort
measures like
calming the infant
after a stressful
procedure, gently
pat or massage the
infant, talking in a
soothing voice,
hold your infant
with as much skin
to skin contact as
possible.

Used divertional
activities by
distracting the
infant with music,
a story or singing a
song.
Provided quiet
environment by
diminishing the
noise, stimulant
and less activity in
the bedside.

Dependent:
Administered
analgesic as
ordered

For the
proper evaluati
on of pain.

The infant is
notable to
verbalize pain
but still, there
are pain clues
that can be
recognize by
the nurse.

To promote
non
Pharmacologica
lpain
management.

To decrease
ones
awareness and
experience of
pain.

To facilitate
comfort, sleep,
and relaxation.

To relive the
pain of the
patient.

Goal met. The
patient is able to
demonstrate
relief of pain from
pain scale 5/5 to
pain scale2/5

VIII. MNAGEMENT
Hirschsprung's disease is treated surgically. The goal is to remove the diseased, nonfunctioning segment of the
bowel and restore bowel function. This is often done in two stages. The first stage relieves the intestinal
obstruction by performing a colostomy. This is the creation of an opening in the abdomen (stoma) through which
bowel contents can be discharged into a waste bag. When the child's weight, age, or condition is deemed
appropriate, surgeons close the stoma, remove the diseased portion of bowel, and perform a "pull-through"
procedure, which repairs the colon by connecting functional bowel to the anus. The pull-through operation usually
establishes fairly normal bowel function.Children with total colonic aganglionosis occasionally fail to benefit from a
pull-through procedure. One option in treating these patients is the construction of an ileoanal S-pouch.
The surgeon may recommend a permanent ostomy if the child has Down syndrome in addition to Hirschsprung
disease, as these children usually have more difficulty with bowel control.
Medical Care
The general goals of medical care are 3-fold: (1) to treat the complications of unrecognized or untreated
Hirschsprung disease, (2) to institute temporary measures until definitive reconstructive surgery can take place,
and (3) to manage bowel function after reconstructive surgery.
Management of complications of recognized aganglionosis is directed toward reestablishing normal fluid and
electrolyte balance, preventing bowel overdistension (with possible perforation), and managing complications,
such as sepsis. Thus, intravenous hydration, nasogastric decompression, and, as indicated, administration of
intravenous antibiotics remain the cornerstones of initial medical management.
Because cardiac malformation (2-5%) and trisomy 21 (5-15%) are associated with congenital aganglionosis,
cardiac evaluation and genetic testing may be warranted.
Colonic lavage, consisting of mechanical irrigation with a large-bore rectal tube and large volumes of irrigant,
may be required.
Balanced salt solutions may help prevent electrolyte imbalances.
Nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may also need to be used in
postoperative patients who develop enterocolitis as a complication. Sodium cromoglycate, a mast cell stabilizer,
has been reported to be of benefit in these patients as well.
Routine colonic irrigation and prophylactic antibiotic therapy have been proposed as a means of decreasing the
risk of enterocolitis.
Injecting the nonrelaxing internal sphincter mechanism with botulinum toxin (BOTOX) has been shown to
induce more normal patterns of bowel movements in postoperative patients with enterocolitis.

Health Teaching
Consultations
Pediatric surgeons
Pediatric gastroenterologists
Geneticists (if trisomy 21 is present)
Diet
The patient should have nothing by mouth before the operation.
Institute tube feeding or formula/breast milk once bowel function resumes.
High-fiber diets and diets containing fresh fruits and vegetables may optimize postoperative bowel function in
certain patients.
Activity
Limit physical activity for about 6 weeks to allow the wound to heal properly (applies more to older children).
Case Study
of
Hirschsprung's
disease



SUB. BY: JASMIN DINGLE
SUB. TO: MRS.SYLVIA LOPEZ