Hirschsprungs disease

What is Hirschsprungs disease?

Hirschsprungs disease is a condition of distal aganglionic bowel that results from the lack of Auerbach (intermuscular) and Meissner (submucosal) plexuses. Functional obstruction of the distal bowel results. Hirschsprung’s disease usually manifests within the first 48 hours of life as failure to pass meconium, or it may manifest with abdominal distention, bilious vomiting, or diarrhea. More than 80% of patients present within the first 6 weeks of life.

What are the X ray findings of Hirschsprungs disease?

The most typical radiographic finding of Hirschsprung’s disease is a dilated colon proximal (upstream) to the distal more diminutive aganglionic segment. Radiographs may also demonstrate high-grade distal bowel obstruction. Radiologic diagnosis of Hirschsprung’s disease requires a contrast enema (water-soluble contrast in almost all circumstances).

Spot radiographs are obtained in the frontal, lateral, and oblique projections. The contrast enema may show the transition zone, which is situated between a narrowed distal aganglionic segment and the distended proximal bowel. The radiographic transition zone may be visualized more distally than the histologic transition zone secondary to stool dilating the proximal part of the aganglionic segment.

Is Hirschsprungs disease diagnosed definitively by imaging?

To make a definitive diagnosis of Hirschsprung’s disease, a rectal (suction) biopsy specimen must demonstrate a lack of ganglion cells. The specificity and sensitivity of a contrast enema for diagnosis are about 70% and 80%, respectively.


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