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Hirschsprung disease

Case contributed by Fazel Rahman Faizi
Diagnosis almost certain

Presentation

Constipation since birth.

Patient Data

Age: 2 years
Gender: Female

Barium enema

Barium

Spot image demonstrates no bowel gas in the rectum.

Small caliber of the rectum is noted. The proximal large bowels are significantly dilated showing loss of normal haustration.

The rectosigmoid ratio is inverted.

Case Discussion

Normally the diameter of the rectum is larger than that of the sigmoid colon. The rectosigmoid ratio is greater than one. In a short segment Hirschsprung disease, this ratio is inverted; the rectum becomes smaller in caliber compared to the sigmoid colon. For a definitive diagnosis, a rectal biopsy should be performed.

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