Hirschsprung's disease by Mr. Bakht Munir Post Graduate College of Nursing Peshawar

1. Post-Basic Speciality
(Pediatric Nursing)
Presentation on
Hirschsprung's Disease
Assigned by
Mdm Bakht Pari
Presented by
Bakht Munir

3. Out Lines
In This Session We Will Discuss & Participants Will Be
Learn The Followings
Basic concept of Hirschsprung's Disease
Pathophysiology
Causes
Signs & symptoms
Prevalence
Risk factors
Investigations
Treatment and
 prognosis of Hirschsprung's Disease

4. History of Hirschsprung's disease
o Hirschsprung's disease first reported in 1691,
by Dutch anatomist Frederik Ruysch
o [However, the disease is named after
o Harald Hirschsprung the Danish physician
who first described two infants who died of
this disorder in 1888

6. Hirschsprung's disease
• Hirschsprung's disease
 Is a congenital disorder of the colon in which
certain nerve cells, known as ganglion cells, are
absent,
The lack of ganglion cells is in the auerbach's
plexus & Meissner plexus
 which is responsible for moving food in the
intestine.
 Causing chronic constipation
The aganglionic segment is narrowed with
normal dilation of proximal colon

7. Segment Involement
Rectum alone is affected in 30% of the cases
Rectosigmoid segment affect 44% of the cases
Entire colon is aganglionic in 10% of the cases
Most common cause of lower intestinal obstruction
in neonates

8. Patho physiology
 Hirschsprung's disease occurs when some of the
nerve cells that are normally present in the intestine
do not form properly while a baby is developing
during pregnancy.
 As food is digested, muscles move food forward
through the intestines in a movement called
peristalsis. When we eat, nerve cells that are present
in the wall of the intestines receive signals from the
brain telling the intestinal muscles to move food
forward.

9. Patho physiology (cont..)
• In children with Hirschsprung's disease, a lack of
nerve cells in part of the intestine interrupts the
signal from the brain and prevents peristalsis in
that segment of the intestine. Because stool
cannot move forward normally, the intestine can
become partially or completely obstructed
(blocked), and begins to expand to a larger than
normal size.
• The problems a child will experience with
Hirschsprung's disease depend on how much of
the intestine has normal nerve cells present.

11. Causes Hirschsprung's Disease
 Between the 4th and the 12th weeks of pregnancy,
 while the fetus is growing and developing
 nerve cells form in the digestive tract
 For unknown reasons
 the nerve cells do not grow in certain points in the
intestine in babies with Hirschsprung's disease

12. Risk Factors
 It occurs five times more frequently in males
than in females
 Children with down syndrome have a higher
risk of having the disease.
 There is possibly a genetic, or inherited, cause
for it

13. Clinical Manifestation
 Failure in new born to pass meconium
 Vomiting , abdominal distention
 Reluctance to feed
 Entero colitis
 Due to ischemic and inflammation
 Perforation and sepsis may fecal masses can felt in
abdomen
 Complete or partial intestinal obstruction
 On PR rectum is empty may flatus and stool as the
finger is withdrawn

14. Clinical Manifestation (cont..)
Children who do not have early symptoms may also
present the following:
 Constipation that becomes worse with time
 Loss of appetite
 Delayed growth
 Passing small, watery, bloody stools
 Loss of energy

16. Prevalence Rate
• Hirschsprung's disease occurs in 1
• Out of every 5,000 live birth
• Four time more common in boys than girls

17. Diagnosis
 Examination:
 Abdominal x-ray.
Obstructions (blockages), and dilated intestine above.
The obstruction
 Barium enema.
A fluid called barium (chemical, chalky, liquid used to
coat the inside of organs so that they will show up on
an x-ray) is given into the rectum as an enema. An x-ray
of the abdomen shows strictures (narrowed areas),
obstructions (blockages), and dilated intestine above.
The obstruction

19. Anorectal manometry.
A test that measures nerve reflexes which are missing
in Hirschsprung's disease.
Biopsy of the rectum or large intestine.
A test That takes a sample of the cells in the rectum
or large intestine and then looks for nerve cells under a
microscope

21. Therapeutic Management
Treatment is surgical with the resection of
aganglionic segment
At times, a child may need to have a colostomy done
so stool can leave the body.
The colostomy may be temporary or permanent
. After a healing period, many children can have the
intestine surgically reconnected above to the anal
opening and have the colostomy closed
A surgical pull-through of ganglionic bowel to the
preanal part is made

22. Pull-Through Procedure
 The surgeon removes the portion of the rectum and
intestine
 that lacks normal nerve cells. When possible, the
remaining portion is then connected to the anal
opening.
 This is known as a pull-through procedure

25. Prognosis
After surgery prognosis is satisfactory
Surgery May Have Some Complications
• Chronic constipation
• Fecal incontinence
• Perineal abscess
• stricture

26. Reference
Basis Of Pediatrics By Pervez Akbar Khan
&
https://www.stanfordchildrens.org/en/topic/default%3Fid%3Dhirschsprungs-
disease-90-
P01999&sa=U&ved=0ahUKEwjd24bV6KHKAhWF2RoKHUASDtgQFggzMAU&us
g=AFQjCNFXTzvi9ari_Xs42kJEphO1Hrgosg