Hirschsprung disease

Case contributed by Mohammad Saboor Rastin


Constipation since birth.

Patient Data

Age: 3 years
Gender: Male

Abdominal x-ray frontal projection in supine position demonstrates marked dilatation of the colon.

No evidence of pneumoperitoneum.


Cologram with administration of diluted barium suspension shows:

  • narrow caliber of the rectum 
  • massive dilatation of the sigmoid colon, descending colon and proximal colon
  • recto-sigmoid ratio appears less than 1

Case Discussion

The above features are suggestive of short segment Hirschsprung disease.

Hirschsprung disease is a condition resulted by aganglionosis (absence of ganglion cells) in the distal colon and rectum.

Among the 4 types, short segment is the most common type and accounts 75% of the cases.

Carefully performed contrast enema helps in diagnosis of the disease, as well as the length of involved bowel segment.

Full-thickness rectal biopsy is needed for confirmation.

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