By : Escel P. Velasquez, RN.

MAN
HIRSCHSPRUNG’S DISEASE:
 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 myenteric plexus of intestine. It is
also known as Megacolon or Congenital Aganglionic
Megacolon.
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)
PATHOPHYSIOLOGY
:
Due to absence of ganglionic cells
There is lack of peristalsis in the affected portion

Functional obstruction of colon

Accumulation of gas & faeces proximal to the defect

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