disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby's colon.

1. HIRSCHSPRUNG
DISEASE

2. HIRSCHSPRUNG DISEASE
• Hirschsprung disease is a congenital condition where there is a lack of nerve
cells in the colon, causing a lack of peristalsis that results in stool being
unable to be pushed through the colon
• Without these nerve cells stimulating gut muscles to help move contents
through the colon, the contents can back up and cause blockages in the
bowel.
• intestinal disorder caused by a lack of ganglion cells in the bowel wall,
principally the colon, which disrupts and abrogates effective peristalsis
• common in Down syndrome
• occurs in 1 of 5000 live births, having a 4:1 male-to-female predominance.
• The majority of cases are sporadic (80%–90%
• will present with meconium plug syndrome (MPS), an obstruction of the
colon by inspissated meconium

4. Signs and Symptoms
• 99% of normal infants pass meconium in the first 24 to 48 hours of life.
• Failure to pass meconium early, feeding intolerance, and abdominal
distention suggest a diagnosis of Hirschsprung’s disease.
• approximately 5% to 10% of affected infants will present with a picture
of enterocolitis (toxic megacolon), characterized by fever, vomiting,
abdominal distention/tenderness, foul-smelling diarrhea, and septic
shock.
• The infant may rapidly deteriorate, with a 50% risk of death if the colon
is not rapidly decompressed

5. Pathophysiology
• During development, neural crest cells (the progenitor or stem cells of the
enteric nervous system) migrate along the intestinal tube to populate the
entire gut in a craniocaudal fashion, with the distal colon, rectum, and
sphincter being the last to be colonized.
• These progenitor cells divide, differentiate, and proliferate to form the
enteric nervous system, of which the ganglion cells are a critical
component.
• Arrest of migration, proliferation, and differentiation results in the
aganglionosis found in Hirschsprung’s disease, which is a relatively
common cause of distal intestinal obstruction in the newborn.
• At the site of arrest, a transition from normal to abnormal innervation is
present, and all intestine distal to this site will be aganglionic and
therefore dysfunctional

6. • Failure to pass stool (meconium)
within the first 48 hours
followingbirth
• abdominal distention
• Abdominal mass
• Ribbon-like or liquid stool
• Sunken eyes
• Pallor
• Dehydration
• Irritable
• Weight loss
• Lethargic
• Transition zone on barium enema
• • Absent ganglion cells and nerve
hypertrophy on rectal biopsy

7. TEST RESULTS/Dx
• Abdominal x-ray: Shows distended areas of the small and large intestines
with little stool in the lower intestine near the anus.
• Rectal biopsy: Absence of nerve ganglion cells in the colon.
• Full-thickness surgical biopsy: Absence of nerve ganglion cells in the colon.
• Suction aspiration of rectum: Absence of nerve ganglion cells in the colon.
• Anorectal manometry: Absence of nerve reflexes.
• Barium enema: Examination of the large intestines shows
strictures/narrowed areas, or intestinal obstructions (blockages), and dilated
intestineabove the blockage.

8. Treatment
• Supportive care to support nutritional intake with temporary ostomy.
• Surgery: After 9 months of age—the affected portion of the colon is
removed.
• if the colon is obstructed, a temporary colostomy or ileostomy is
performed to decompress the colon.
• Once decompressed, a second surgery is performed to remove the
affected portion of the colon and remove the colostomy or ileostomy.

9. General goals of medical care
1. to treat the manifestations and complications of untreated
Hirschsprung disease,
2. to institute temporizing measures until definitive reconstructive
surgery, and
3. to manage the postoperative bowel function.

10. • Two types of surgery :- a pull-through surgery
or an ostomy surgery.
Pull-through surgery
• In this the lining of the diseased part of the
colon is stripped away.
• Then, the normal section is pulled through the
colon from the inside and attached to the anus.
• This is usually done using minimally invasive
(laparoscopic) methods, operating through the
anus.

11. Tx
Ostomy surgery
• In children who are very ill, surgery might be done in two steps.
• the abnormal portion of the colon is removed and the top, healthy
portion of the colon is connected to an opening the surgeon creates in
the child's abdomen.
• Stool then leaves the body through the opening into a bag that
attaches to the end of the intestine that protrudes through the hole in
the abdomen (stoma)
• This allows time for the lower part of the colon to heal.

12. Nursing intervention
Preoperatively:
• Nothing by mouth.
• Administer IV fluids as ordered to maintain fluid and electrolyte balance.
• Insert a nasogastric (NG) tube to decompress the upper GI tract.
• Administer normal saline or mineral oil enemas to clean the bowel.
• Administer antibiotics as ordered.
• broad-spectrum antibiotics, and correction of acid–base deficits
and electrolyte abnormalities are promptly initiated

13. Nursing intervention …
Postoperatively:
• Strict input and output measurement to monitor fluid levels closely.
• Provide care for the colostomy or ileostomy, if necessary.
• Monitor bowel sounds.
• Begin feeding by mouth when bowel sounds are present.
• Nothing should be placed in the rectum.
• Monitor for constipation.
Explain the disorder and treatment to the family and instruct them on the proper
care for the wound and how to care for the colostomy or ileostomy, if necessary.
Tell the family to call the health-care provider at the first signs of constipation,
dehydration, fever, vomiting, or diarrhea

14. Complications and Prognosis
• Early complications of the pull-through operation include inadequate
blood supply to the coloanal anastomosis, anastomotic stricture,
anastomotic dehiscence, and cuff abscess.
• Later complications include Hirschsprung’s-associated enterocolitis,
perianal skin excoriation, and recurrent constipation.
• Diarrhea, Constipation, Leaking stool (fecal incontinence), Delays in
toilet training
• The infant usually thrives postoperatively and grows normally.
• It is not uncommon for the infant to have frequent stools during the
immediate postoperative period, which gradually normalize in frequency.