Presentation
Constipation since birth.
Patient Data
Age: 3 years
Gender: Male
From the case:
Hirschsprung disease
Frontal
Abdominal x-ray frontal projection in supine position demonstrates marked dilatation of the colon.
No evidence of pneumoperitoneum.
From the case:
Hirschsprung disease
Lateral
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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