Hirschsprung disease on a contrast enema

Fluoroscopy · X-ray

First and second year — the floor first, then every step

In a neonate with delayed meconium or distal obstruction, the contrast enema finds the transition zone and the rectosigmoid ratio — the diagnosis is made on rectal biopsy, but the enema tells the surgeon where the aganglionic segment ends.

Orient first

  • Aganglionosis starts at the anus and extends proximally; the aganglionic segment is narrow and the normal bowel above it dilates — the transition zone is between them.
  • The rectum is normally the widest part of the distal colon (rectosigmoid ratio above 1); a ratio below 1 supports Hirschsprung disease.
  • Differential in a neonate with distal obstruction: meconium plug / small left colon, meconium ileus (cystic fibrosis), ileal or colonic atresia, anorectal malformation.

Acquire the study

  • Water-soluble contrast enema, early lateral projections of the rectum, then frontal views; a 24-hour delayed radiograph if the diagnosis is uncertain.

The manoeuvre

  • Early lateral projection during filling: rectal calibre against the sigmoid (rectosigmoid ratio).
  • Transition zone: a cone or abrupt change from narrow distal to dilated proximal bowel — note its level.
  • Irregular, saw-tooth contractions of the aganglionic segment.
  • Delayed radiograph at 24 hours: retained contrast supports the diagnosis.
  • Total colonic disease: a short, featureless colon (microcolon or normal-calibre colon) with no transition.

What confirms it

  • A transition zone with a reversed rectosigmoid ratio — to be confirmed by rectal suction biopsy.

What licenses you to exclude it

  • A normal enema does not exclude Hirschsprung disease (ultrashort or total colonic forms) — biopsy decides.

The classic misread

  • A rectal examination or washout before the enema can hide the transition zone.

See it on real cases

Direct links to Radiopaedia — the reference article and worked cases with their images. Each opens on Radiopaedia.

Key papers

Reviews and guidelines from RSNA, ESR and related journals. Each opens at its DOI.

  1. Radiologist performance in the interpretation of contrast enemas performed for Hirschsprung's disease in children >1 year of age ↗Hwang TJ, Servaes S, Mattei P, et al. · Clinical Radiology 2017RCR · PubMed
  2. Visualisation of the rectoanal inhibitory reflex with a modified contrast enema in children with suspected Hirschsprung disease ↗Vult von Steyern K, Wingren P, Wiklund M, et al. · Pediatric Radiology 2013SPR · ESPR · PubMed
  3. Diagnosis of Hirschsprung disease by hydrocolonic sonography in children ↗Ma Y, Jiang Q, Zhang Z, et al. · European Radiology 2022ESR · PubMed

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