Hirschsprung Disease involves a failure of nerve cells to migrate into the large intestine during development, resulting in constipation. Diagnosis involves imaging tests like plain abdominal x-rays and barium enemas to show dilation of the colon above a narrowed segment. Anorectal manometry demonstrates failure of the internal anal sphincter to relax in response to rectal distension. Biopsy of the rectal wall is the most accurate diagnostic test. Treatment depends on age and severity, and may involve resuscitation, a temporary colostomy to decompress the bowel, and definitive surgery to remove the affected segment.

1. Hirschsprung Disease
Diagnosis & Treatment
By SMS

2. Plain abdominal radiograph
show dilated loops of
bowel with air-fluid levels.
Occasionally, there may
be a small amount of air in
the undistendedrectum
and the dilated colon
above it.

3. Barium Enema
• Done on an unprepared colon
• There should not have been a
digital rectal examination or rectal
washout for 3 days prior to the
examination.
• The enema will demonstrate the
flow of barium from the
undilatedrectum through a funnel-
shaped transitional zone into the
proximal dilated colon.

4. Anorectal Manometry
• This demonstrates the failure of the internal anal sphincter to relax when the
rectum is distended -the recto-anal reflex
• The pressure in the anal canal is not reduced on inflating the balloon in the
rectum.
• The test is reliable only in the older child

5. Biopsy Studies
• Is the most accurate way of
• diagnosing Hirschsprung' s disease.
• This biopsy is most often taken from the posterior wall of the rectum 1, 2, and
3cm above the dentate line under general anaesthesia.
• The biopsy specimen should be full thickness, containing mucosa,
submucosaand muscle wall.
• CBC
• Elecrtolyte, urea and creatinine
• Grouping/ Cross-matching

6. Treatment
•Depends on mode & age at presentation,length of the involved
segment , severity of symptoms
• Acute intestinal obstruction
• Resuscitation
• •IV LINE
• •NG TUBE
• •URETHRAL CATHETER
• •BROAD SPECTRUM ANTIBIOTICS

7. Txt cont…..
• A temporary colostomy is done
• Site of colostomy should be as low as possible in the ganglionatedsegment.
• However, in the abscenceof complications, a defunctioningcolostomy to
prevent enterocolitis.
• The colostomy is sited in normal bowel usually just above the transitional
zone.
• Definitive surgery is done when the infant is 10kg.
• If a colostomy cannot be done or is unacceptable, regular saline rectal
washouts until the infant is ready for definitive operation.

8. Txt in Older children
Definitive operation is performed in the older child presenting for the first time
if there is no enterocolitis.
• Swenson's Operation
• The transitional zone is resected with the aganglionic segment and the
normal bowel pulled down and anastomoses to a cuff of anus 2-3cm above
the anal
• Duhamel's Operation
• Soave's Operation
verge.

9. THANKS