Its a pediatric disease which is very severe, this content will be useful for the students who pursuing nursing course

1. HIRSCHSPRUNG’S DISEASE:

2. HIRSCHSPRUNG’S DISEASE:
Definition:
Hirschsprung’s disease is a disorder of the gut caused
due to congenital absence of ganglion cells in the
submucosal and myentric plexus of intestine. It is
also known as Megacolon or Congenital Aganglionic
Megacolon.

3. INCIDENCE & ETIOLOGY:
1 in 5000 live births, it is more common in males than
females.
Through research it is suggested that it is caused by
interaction between two proteins encoded by two
genes:
 RET Proto- oncogene (chromosome 10)
 EDNRB gene (chromosome 13)

4. PATHOPHYSIOLOGY:
Enlargement of the colon occurs and called Megacolon
Accumulation of gas & faeces proximal to the defect
Functional obstruction of colon
There is lack of peristalsis in the affected portion
Due to absence of ganglionic cells

5. CLINICAL FEATURES:
In neonates and infants:
 Failure to pass meconium
 Abdominal distension
 Bile- stained vomiting
 Shock
 Episodes of diarrhea & constipation
In older children:
 Constipation with abdominal distension
 When stool passed foul smelling, and liquid in consistency
 Malnourished and anemic

6. DIAGNOSTIC EVALUATION:
Hirschsprung’s disease is suspected in a baby who
has not passed meconium within 48 hours of birth.
Rectal examination
Palpation
Anorectal manometry
Barium enema & Rectal biopsy

7. MANAGEMENT:
A. Medical management:
• Administration of Isotonic enema
• Administration of stool softeners
• Low residue diet
B. Surgical management:
The surgery involves two steps:
1) TEMPORARY COLOSTOMY
2) DEFINITIVE SURGERY, after about a year of these
surgeries, the colostomy is closed.

8. A): Swenson Procedure
B): Duhamel Procedure
C): Soave’s Procedure

9. Swenson & Duhamel Procedure:

10. C): Soave’s Procedure:

11. NURSING MANAGEMENT:
Pre- operative care:
i. Assessment, complete history of new born.
ii. After diagnosis, nurse must help parents.
iii. Nurse taught about giving isotonic enema,
suppositories and stool softeners.
iv. Low residue diet must be given to the child.
v. Monitor vital signs & abdominal girth of child.
vi. Keep the child in semi-fowlers postion.
vii. Withhold oral feeds & Nasogastric aspiration done
on the night before surgery.

12. Post-operative care:
i. Monitor vital signs, observe abdominal bleeding.
ii. Place the child in comfortable position according
to the physician order.
iii. Child is NPO, so administer IV fluids as ordered.
iv. Monitor bowel sound.
v. Colostomy care is to be done which includes
following:
 Observe stoma for its colour (reddish- pink colour)
 Observe for bleeding, purulent drainage, edema.
 Provide bland diet.
 apply zinc oxide ointment on skin around stoma.

13.  Frequently empty the collecting bag.
 Keep the colostomy clean and dry.
 Educate parents about colostomy care.
vi. Encouraging and supporting the family during this stressful
time is the key nursing intervention.