HIRSCHPRUNGS DISEASE &

MALROTAION AND VOLVULUS

&
NECROTIZING ENTEROCOLITIS

Presented by: Maqsooda sttar

Lecturer, snc
RN, RM, Post RN BSN
Dip in paediatric health nursing
 Objectives

At the end of session the learner will be able to

 Describe Hirschsprung,s disease, Malrotaion & volvulus,
Necrotizing enterocolitis &
Their causes sign/symptoms, diagnosis ,medical and nursing
management
 HIRSCHSPRUNG DISEASE
(AGANGLIONIC MEGACOLON)
 HIRSCHSPRUNG DISEASE
 Aganglionic megacolon, also called hirschsprung
disease, is characterized by persistent constipation
resulting from partial or complete intestinal obstruction of
mechanical origin.

 In some cases, aganglionic megacolon or hirschsprung

disease may be severe enough to be recognized
 INTRODUCTION
 Congenital aganglionic megacolon, also
called Hirschsprung disease, is characterized by persistent
constipation resulting from partial or complete intestinal
obstruction of mechanical origin.
 In 1886, Harold Hirschsprung first described Hirschsprung
disease as a cause of constipation in early infancy.
 Early recognition and surgical correction of Hirschsprung
disease protect affected infants from enterocolitis and
debilitating constipation.
STATISTICS AND INCIDENCES

 Cases of aganglionic megacolon is seen in approximately 1

per 5000 live births.
 Prevalence may vary by region and has been shown to be as
high as 1 per 3000 live births, The overall mortality of
Hirschsprung enterocolitis is 25-30%, which accounts for
almost all of the mortality from Hirschsprung disease.
 Hirschsprung disease is approximately 4 times more
common in males than females.
 Conti…
 Nearly all children with Hirschsprung disease are diagnosed
during the first 2 years of life.
 Approximately one-half of children affected with this
disease are diagnosed before they are aged 1 year; a small
number of children with Hirschsprung disease are not
recognized until much later in childhood or adulthood.
 CAUSES
The causes of aganglionic megacolon are:
 Genetic causes.
 Associated conditions. Hirschsprung disease is strongly
associated with
 down syndrome; 5-15% of patients with hirschsprung
disease also have trisomy 21;
 other associations include
 waardenburg syndrome,
 congenital deafness,
 malrotation,
waardenburg syndrome
 gastric diverticulum, and intestinal atresia.
 Clinical Manifestations
 Examination of infants affected with HIRSCHSPRUNG disease
reveals:
 Some infants with Hirschsprung disease have signs and symptoms
of intestinal obstruction shortly after they are born, which
include7,8
 not having a first bowel movement within 48 hours after birth
 green or brown vomit
 swollen abdomen, or belly
 problems feeding
 explosive stools or diarrhea after a doctor inserts a finger into the
newborn’s rectum
 Newborns with Hirschsprung disease may also have signs or
symptoms of complications.
 Chronic constipation. Older infants and children with
Hirschsprung disease usually present with chronic constipation .
conti…

 Absence/delayed passage of meconium. During the

newborn period, infants affected with Hirschsprung disease
may present with failure of passage of meconium.
 Vomiting. Repeated vomiting is present due to intestinal
obstruction.
 Malnourishment. Poor nutrition results from the early
satiety, abdominal discomfort, and distention associated
with chronic constipation.
 DIAGNOSIS
 The diagnosis of aganglionic megacolon is made through the
following data:
 Laboratory studies. CBC count, order this test if
Enterocolitis is suspected; elevation of WBC count
 Plain abdominal radiography. Perform this test with any
signs or symptoms of abdominal obstruction.
 Rectal biopsy. Diagnosis is confirmed through rectal biopsy.
 Rectal manometry. In older children who present with
chronic constipation and an atypical history for either
Hirschsprung disease or functional constipation, anorectal
manometry can be helpful in making or excluding the
diagnosis.
 MEDICAL MANAGEMENT
Treatment involves:
 Initial therapy. If a child with Hirschsprung disease has
symptoms and signs of a high-grade intestinal obstruction,
initial therapy should include intravenous hydration, with
holding 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.
 PHARMACOLOGIC MANAGEMENT
 Drug therapy currently is not a component of the standard
of care for this disease itself; however, some medications
may be used to treat complications of Hirschsprung
disease.
 Antibiotics. Administer broad-spectrum antibiotics to
patients with enterocolitis.
 SURGICAL MANAGEMENT
 surgical options vary according to the patient’s age, mental
status, ability to perform activities of daily living, length of
the aganglionic segment, degree of colonic dilation, and
presence of enterocolitis.
 NPO the patient
 Nursing Management

 Nursing care management for a child with aganglionic

megacolon include:
 Nursing Assessment
Assessment involves:
 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.
 Physical examination. During the physical exam, observe
for distended abdomen and signs of poor nutrition; record
weight and vital signs
 NURSING DIAGNOSIS
 Based on the assessment data, the major
nursing diagnoses for Hirschsprung disease are:
 Constipation related to decreased bowel motility.
 Imbalanced nutrition: less than body requirements related to
anorexia.
 Fear (in the older child) related to impending surgery.
 Risk for impaired skin integrity related to irritation from the
colostomy.
 Acute pain related to the surgical procedure.
 Deficient fluid volume related to postoperative condition.
 Impaired oral and nasal mucous membranes related to NPO
status and irritation from NG tube.
 Deficient knowledge of caregivers related to understanding
of postoperative care of the colostomy.
 NURSING CARE PLANNING AND
GOALS
 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.
 NURSING INTERVENTIONS
Nursing interventions for a child with aganglionic
megacolon include:
 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 with skin-
toughening preparations that strengthen it and provide better
adhesion of the appliance.
 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.
 Conti…
 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
 Provide oral and nasal care. Perform good mouth care at least
every 4 hours; at the same time , Clean your child's skin around the tube
often with warm water, removing any secretions. If the nostril is reddened or the
skin is irritated, remove the tube and replace it in the other nostril, if possible.
 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.
 EVALUATION
 Goals are met as evidenced by:
 The child’s skin integrity is maintained.
 The child’s behavior indicates minimal pain.
 The child’s fluid intake is adequate.
 The child’s oral and nasal mucous membranes remain intact.
 The family caregivers demonstrated skill and knowledge in
caring for the colostomy.
 DOCUMENTATION
 Documentation in a patient with aganglionic megacolon
include:
 Individual findings, including factors affecting, interactions,
nature of social exchanges, specifics of individual behavior.
 Intake and output.
 Signs of infection.
 Cultural and religious beliefs, and expectations.
 Plan of care.
 Teaching plan.
 Responses to interventions, teaching, and actions performed.
 Attainment or progress toward desired outcome.
 Practice Quiz: Aganglionic Megacolon
1. The nurse is assessing an infant with Hirschsprung’s
disease. The nurse can expect the infant to:
 A. Weigh less than expected for height and age.
B. Have a scaphoid-shaped abdomen.
C. Exhibit clubbing of the fingers and toes.
D. Have hyperactive deep tendon reflexes.
 1. Answer: B. Have a scaphoid-shaped abdomen.
 Option B: The child with Hirschsprung’s disease will have a
scaphoid or hollowed abdomen.
 Options A, C, and D: Answers A, C, and D do not apply to the
condition; therefore, they are incorrect.
2.. Dustin who was diagnosed with Hirschsprung’s disease
has a fever and watery explosive diarrhea. Which of the
following would Nurse Joyce do first?
 A. Administer an antidiarrheal.
B. Notify the physician immediately.
C. Monitor the child every 30 minutes.
D. Nothing. (These findings are common in Hirschsprung’s disease.)
2. Answer: B. Notify the physician immediately
 Option B: For the child with Hirschsprung’s disease, fever and explosive
diarrhea indicate enterocolitis, a life-threatening situation. Therefore, the
physician should be notified directly.
 Option A: Generally, because of the intestinal obstruction and inadequate
propulsive intestinal movement, antidiarrheals are not used to treat
Hirschsprung’s disease.
 Option C: The child is acutely ill and requires intervention, with
monitoring more frequently than every 30 minutes.
 Option D: Hirschsprung’s disease typically presents with chronic
constipation.
3. Parents bring their infant to the clinic, seeking treatment
for vomiting and diarrhea that has lasted for 2 days. On
assessment, the nurse in charge detects dry mucous
membranes and lethargy. What other findings suggest a
fluid volume deficit?
 A. A sunken fontanel
B. Decreased pulse rate
C. Increased blood pressure
D. Low urine specific gravity
 3. Answer: A. A sunken fontanel.
 Option A: In an infant, signs of fluid volume deficit (dehydration
) include sunken fontanels, increased pulse rate, and decreased
blood pressure.
 Options B and C: These options are the opposite of the
symptoms in fluid volume deficit.
 Option D: This is not a symptom of fluid volume deficit.
4. A newborn’s failure to pass meconium within the first 24
hours after birth may indicate which of the following?
 A. Hirschsprung disease
B. Celiac disease
C. Intussusception
D. Abdominal wall defect
4. Answer: A. Hirschsprung disease.
 Option A: Failure to pass meconium within the first 24 hours after birth
may be an indication of Hirschsprung disease, a congenital anomaly
resulting in mechanical obstruction due to inadequate motility in an
intestinal segment.
 Options B, C, and D: Failure to pass meconium is not associated with
celiac disease, intussusception, or abdominal wall defect.
5. Which of the following electrolytes are lost as a result of
vomiting?
 A. bicarbonate and calcium
B. sodium and hydrogen
C. sodium and potassium
D. hydrogen and potassium
5. Answer: D. hydrogen and potassium.