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Hirschsprung disease

Case contributed by Dr Tee Yu Jin

Presentation

Known Hirschsprung disease, biopsy-proven during the first month of life. The child was feeding well with good regular bowel output and was treated conservatively. Currently presented with abdominal distension.

Patient Data

Age: 5 months
Gender: Male
X-ray

Distension of bowel loops at the central part of the abdomen.

Barium

There is a smooth flow of barium from the anal canal until the proximal sigmoid colon. The rectum is small in caliber with dilated sigmoid (rectosigmoid ratio less than 1.0). There is a transition zone at the distal sigmoid colon with dilatation of the proximal bowel (annotated on the film).

Annotated image

The annotated image demonstrates the transition zone with small caliber rectum and dilated sigmoid colon (rectosigmoid ratio less than 1.0).

Case Discussion

Features of Hirschsprung disease with a transition zone in the distal colon. 

Hirschsprung disease is an intestinal motor disorder characterized by the absence of Auerbach and Meissner plexuses of the distal GI tract. Lower GI contrast study demonstrates a rectosigmoid ratio of <1.0. However, the depicted transition zone on the contrast enema is not accurate at determining the transition between absent and present ganglion cells.

Case courtesy of Dr Che Zubaidah Bt Che Daud, Consultant Pediatric Radiologist of the Women and Children Hospital, Kuala Lumpur, Malaysia.

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Case information

rID: 71552
Published: 11th Oct 2019
Last edited: 14th Mar 2020
Inclusion in quiz mode: Included

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