Penyakit Hirschprung

Hirschprung Disease
• Kelainan kongenital akibat kegagalan
migrasi krista neuralis ke colon.
• Tidak terbentuk sel ganglionik pd
plexus myentericus (Auerbach) dan
plexus submucosal (Meissner)
• 80%  rectosigmoid
• Klinis :
– Delayed meconium (>24h)
– Abdominal distention
– Bilous vomiting
– Severe diarrhea alternating with
constipation
• Dx :
– Barium enema
– Rectal biopsy
– Anorectal manometry
Sign and Symptoms

• Symptoms may recur after previously

resolving with laxatives, or feeding
changes.

• Digital Rectal examination may

demonstrate a tight anal sphincter
and explosive discharge of stool and
gas.
- Frog-like abdomen
- Darm contour
- Darm steifung
- Metallic sound
 Radiographic Features
• Imaging can help diagnose • Contrast barium enema radiographs,
Hirschsprung’s disease. A plain After the dilation process begins, the
abdominal radiograph may show diseased portion of the colon will
a dilated small bowel or proximal appear normal and the more proximal
colon (no air in the rectum) colon will be dilated. A “transition zone”
(the point where the normal bowel
becomes aganglionic) may be visible on
a contrast enema radiograph