Hirschsprung's disease (or megacolon) is a rare genetic disease that affects 1 in 5,000 births, mostly boys (80%). These are often sporadic forms (with no family history) but familial forms are known.

> A functional occlusion

This disease is characterized by the absence of nerve ganglion cells in the wall of the terminal segment of the intestine (“aganglionary” segment). This results in intestinal paralysis, generally resulting in a functional obstruction, sometimes simply in significant constipation. There are different forms of

Hirschsprung's disease, the severity of which depends on the length of the aganglionic segment.

In 80% of cases, it is the “classic” form which simply affects the end of the colon (the rectum and the sigmoid colon). But there are “long” and “total” forms and sometimes associated anomalies.

> A suitable surgical intervention

The diagnosis is usually made in the very first weeks of life. The babies are then taken care of by a pediatric surgeon who will decide on the appropriate intervention.

This operation consists of removing the aganglionic segment, it is less and less often preceded by a temporary placement of the intestine to the skin (enterostomy), generally at the level of the colon (colostomy). Its goal is to restore intestinal and anorectal function as normal as possible.