HIRSCHSPRUNG’S DISEASE (Aganglionic Megacolon)

Definition
• Hirschsprung’s disease is a congenital anomaly that results in
mechanical obstruction from inadequate motility of part of the
intestine.*
• HD occurs when there is blockage in the large intestine that is
caused by missing nerve cells in the colon. The colon stays
contracted and no digested food can be pushed through
allowing intestinal content to build up
• HD develops in children before they are born. It is not caused
by anything the mother did while pregnant.
• HD affects 1 in 5000 live births*
Etiology
• Absence of special nerve cells in the intestine called ganglion
cells at the last part of the large intestine.*
• Some appears to be due to autosomal dominant inheritance*

Pathophysiology
 HD develops before a child is born. Normally, nerve cells grow
in the baby's intestine during the 5th and 12th weeks of
gestation. These nerve cells grow down from the top of the
intestine all the way to the anus. With HD, the nerve cells stop
growing before they reach the end resulting in a loss of the
rectosphincteric reflex. The ability of the internal sphincter to
relax decreases thus resulting to lack of peristalsis. Normally
when the stool bolus enters the rectum, the internal sphincter
relaxes and the stool is evacuated. In HD the internal sphincter
does not relax.
Types
Long-segmented HD
 ganglion cells are missing from the whole large intestine and
even part of the small intestine
Short-segmented HD
 ganglion cells are missing from only part of the large intestine
Signs and Symptoms
• failure to pass meconium within the first 48 hours
• abdominal distention
• vomiting
• constipation
• acute intestinal obstruction
• ribbonlike, foul-smelling stools
• slow growth and development
 • chronic constipation

Diagnostic exams
Plain abdominal x-ray
 confirms intestinal obstruction
Barium enema x-ray
 the doctor puts barium through the anus into the intestine
before taking the picture. Barium is a liquid that makes the
intestine show up better in the x-ray
Anorectal Manometry
 the doctor inflates a small balloon inside the rectum. Normally,
the anal muscle will relax. If it doesn’t, HD may be is the
problem*
Rectal Biopsy
 the doctor removes and looks at a tiny piece of the intestine
under a microscope. If the nerve cells are missing, HD is the
problem.*
Management
 *Help the parents adjust to the congenital disorder*
 Foster infant-parent bonding
 Prepare the parents for the medical-surgical interventions
 Pull-through surgery
 Is the removal of the aganglionic portion of the intestine
and connecting the healthy part to the anus
 Ostomy
 The doctor takes out the diseased part of the intestine
and cuts out a small hole in the baby’s abdomen.* Then
the doctor connects the top part of the intestine to the
stoma.* Stool goes into a bag attached to the skin around
the stoma.*
 Teach the caregivers on how to care for the stoma, how to
change the bag that collects the stool and talk about their
worries
 Measure the intake and output, including the NG tube losses
and stool from the ostomy
 Monitor IV fluids to maintain adequate hydration and
electrolyte balance
 Monitor the return of the bowel sounds and passage of stool*
 Advice patient to drink plenty of water and inform the
caregivers the importance of liquids specially after surgery
 Advice patient to eat high-fiber food
Nursing Diagnosis
• Altered elimination pattern: constipation r/t reduced bowel
function
• Risk for infection r/t inflammation of the intestine
• Imbalance nutrition less than body requirement: slow growth
and development r/t malabsorption of nutrients
• Risk for infection r/t inadequate primary defenses
• Risk for diarrhea r/t bowel incontinence