Hirschsprung disease (HD) is a disorder of the gut which is caused by the failure of the neural crest cells (enteric ganglion cells) to migrate completely during fetal development of the intestine. The affected segment of the colon fails to relax, causing an obstruction. In the majority of affected people, the disorder affects the short segment of the distal colon. In rare cases the aganglionosis involves more of the colon. In 5 percent of cases the entire colon is affected. Hirschsprung is also sometimes called congenital aganglionic megacolon.
Hirshsprung disease occurs in approximately one in 5000 births. (from wikipedia)
Image from Praanadah Specialty Hospital (link)
Hirschsprung disease (HD) can be diagnosed by means of contrast enema examination, which can show the presence of a transition zone, irregular contractions, mucosal irregularity, and delayed evacuation of contrast material.
Transition zone is the term applied to the region in which a marked change in caliber occurs, with the dilated, normal colon above and the narrowed, aganglionic colon below; failure to visualize a transition zone does not rule out the presence of the disease.
If Hirschsprung’s is suspected, then rectal suction biopsies should be performed to confirm diagnosis.
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