Hirschsprung's disease is a disorder of the gut caused by the congenital absence of ganglion cells in the intestine. It occurs in around 1 in 500 live births and is more common in males. Symptoms in neonates include failure to pass meconium and abdominal distension. In older children, symptoms include constipation and foul-smelling stools. Diagnosis involves tests like barium enema and rectal biopsy. Treatment consists of medical management with enemas or surgery to remove the affected segment and reconnect the intestine. Nursing care focuses on monitoring for complications pre-and post-surgery and providing bowel care.

1. HIRSCHSPRUNG’S
DISEASE

2. DEFINITION
• It a disorder of the gut caused due to
congenital absence of ganglion cells in
the submucosal and myenteric plexus of
intestine.
• Also known as Megacolon or congenital
aganglionic Megacolon.

3. INCIDENCE
• Occurs in 1 in 500 live births.
• More common in males than females.

4. PATHOPHYSIOLOGY
Due to absence of ganglionic cells.
Lack of peristalsis in the affected portion.
Functional obstruction of colon.
Accumulation of gas and faeces proximal to the defect.
Enlargement of the colon.

5. CLINICAL FEATURES
In neonates and infants……
• Failure to pass meconium.
• Abdominal distension within 1-2 days after birth.
• Bile stained vomiting.
• Enterocolitis due to fecal stagnation.
• Enterocolitis may lead to dehydration and sepsis.

6. In older children
• Constipation with abdominal distension due to mass of faeces and gas.
• Foul smelling stools.
• Ribbon like stools.
• Protruding abdomen.

7. DIAGNOSTIC EVALUATION
1. Suspected in babies who have not passed meconium with 48 hours of
birth.
2. On palpation fecal mass is felt in left lower portion of the abdomen.
3. Barium enema.
4. Rectal biopsy: shows absence of ganglion cells in the mucosa.

8. MANAGEMENT
Medical management:
1. Administration of enema.
2. Administration of stool softeners.
3. Low residue diet.

9. Surgical management
• The aim of surgery is to remove the aganglionic bowel followed
by anastomosis of the remaining portion.
Done in two stages…….

10. Stage I:
• Temporary colostomy is done in the sigmoid or transverse
colon.
• It enables normal distal bowel movements to return to original
tone and size.

12. Stage II:
• Involves definitive surgery done when child’s weight and condition is
appropriate.
• Involves excision of aganglionic segment and anastomosis between
ganglionic colon and anus.
• Common definitive surgeries……
Swenson procedure
Soave’s procedure
Duhamel procedure

13. NURSING
MANAGEMENT

14. PRE-OPERATIVE NURSING CARE
• Assess bowel movements.
• Assess for passage of meconium and vomiting.
• Give enema, stool softeners.
• Provide low residue diet.
• Monitor vital signs.
• Monitor abdominal girth.
• Give semi fowlers position.

15. • Withhold oral feeds and NG aspiration is done a night before surgery.
• Repeat saline enema and bowel wash with antibiotic solution.
• Observe the child for preoperative complications like dehydration,
shock, etc.
PRE-OPERATIVE NURSING CARE

16. POST OPERATIVE NURSING CARE
• Monitor vital signs.
• Observe surgical site for bleeding.
• Monitor for abdominal distension.
• Administer IV fluids.
• Monitor for return of bowel sounds.
• Provide colostomy care.
• Keep surgical site clean.