HIRSCHSPRUNG'S

DISEASE
NCM 109
Presented by:
Patricia Isabel Dumaguin
Mariah Alexis Encina
 HIRSCHSPRUNG’S
DISEASE
Hirschsprung’s disease (HD), also
known as congenital aganglionic
megacolon disease, is a congenital
disease in which ganglionic cells fail
to develop in the large intestine.
This commonly presents as delayed
or failed passage of meconium
around birth.

“Hirschsprung's disease - Pathophysiology - TeachMePaediatrics.” Teach Me Paediatrics, https://teachmepaediatrics.com/surgery/abdominal/hirschsprungs-disease/. Accessed 13 April 2022.
 STATISTICS
The international incidence of Hirschsprung
disease is believed to be 1 case per 1,500-
1,700.
Males have a higher incidence to female. There
are several genes that are believed to be
involved. The main genes that are associated
with the disease are those that encode the
proteins for the RET signaling pathway and
endothelin type B receptor pathway.
“Hirschsprung's disease - Pathophysiology - TeachMePaediatrics.” Teach Me Paediatrics,
https://teachmepaediatrics.com/surgery/abdominal/hirschsprungs-disease/. Accessed 13 April 2022.
https://www.youtube.com/watch?v=1pylBKNMcnw
PATHOPYSIOLOGY
Migration failure of neural crest
cells
Absence of the parasympathetic
ganglion cells
Absence of peristalsis in the
aganglionic colon
Functional obstruction
COMPLICATION
Serious intestinal infection called
enterocolitis
soiling, or when a child has accidental
bowel movements in their underwear
megacolon, or severe constipation and
swelling of the intestine
malnutrition, or when a child doesn’t get
enough vitamins, minerals, and other
nutrients to grow and be healthy
perforation, or a hole in the wall of the
intestine, which is more common in
newborns than in older children
HTTPS://WWW.NIDDK.NIH.GOV/HEALT
H-INFORMATION/DIGESTIVE-
DISEASES/HIRSCHSPRUNG-
DISEASE/DEFINITION-FACTS
PREVENTION
According to the Trusted medical
advice from the American Academy
of Family Physicians
(familydoctors.org), you cannot
prevent or avoid it. However,
parents who have the disease can
unknowingly pass it on to their
children.

HTTPS://FAMILYDOCTOR.ORG/CONDITION/HIRSCHSPRUNGS-
DISEASE/#:~:TEXT=CAN%20HIRSCHSPRUNG'S%20DISEASE%20BE%2
0PREVENTED,IT%20ON%20TO%20THEIR%20CHILDREN.
 MANAGEMENT
Initial management would involve IV
antibiotics, nasogastric tube insertion and
bowel decompression.
The only definitive treatment is surgery.
There are two ways this can be done: a pull-
through surgery or an ostomy surgery.
1. https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/treatment
 Pull-through surgery is the primary treatment for
Hirschsprung disease to remove the affected area of the bowel

https://surgery.wustl.edu/ask-the-doctor-hirschsprung-disease-with-dr-baddr-shakhsheer/
 An ostomy is performed to allow patients to pass digestive
waste as the bowel heals from Hirschsprung disease

1. https://surgery.wustl.edu/ask-the-doctor-hirschsprung-disease-with-dr-baddr-shakhsheer/
 MANAGEMENT
After surgery, it is still important to establish protocol in
diet and activity to promote a healthy digestive system
during recovery and throughout life for those effected by
HD.
For infants, this includes formulas to relieve constipation,
increased fluids and laxatives as directed by a physician.
For young children through adults, including high-fiber
foods and physical activity will promote regular bowel
movements.
1. https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/treatment
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