Chronic Constipation and Encopresis in Children

  Hirschsprung's disease

Hirschsprung's disease is sometimes called congenital megacolon or congenital intestinal aganglionosis.  Hirschsprung's disease is a rare disorder occurring in about 1 out of every 5,000 to 10,000 newborn babies (by way of comparison, constipation occurs in as many as 1 out of every 5 to 10 children). This disease is much more common in boys than girls.

Hirschsprung's disease results when some of the nerves in the intestine (ganglion cells) don't develop normally. These nerves are important in helping the intestine relax. In children with Hirschsprung's disease, the intestine is constantly squeezed tight, preventing stool from passing. Almost all children with Hirschsprung's disease have problems with constipation from the day they are born; as many as half of babies with Hirschsprung's disease will not pass their first bowel movement during the first 36 hours of life.

We still don't know what causes Hirschsprung's disease.  While genetics probably play a role, most parents cannot identify anybody in their family with Hirschsprung's disease.  There is currently no evidence to indicate that Hirschsprung's disease is caused by any medications or exposures to toxins during pregnancy.

be2.gif (37987 bytes)Most of the time, when a doctor is concerned about the possibility of Hirschsprung's disease, he or she will have a barium enema performed.  This is an x-ray procedure.  The radiologist inserts a small tube into the baby's bottom and pumps barium (an inert dye) into the lower intestine.  The radiologist is looking for an area of narrowing that is suggestive of Hirschsprung's disease (see the picture at the right).  If the barium enema is suggestive of Hirschsprung's disease, a rectal biopsy is usually performed.  A small piece of the lower intestine is removed and examined under the microscope for the presence or absence of ganglion cells.  If ganglion cells are present, the baby doesn't have Hirschsprung's disease.  Sometimes an anorectal manometry will be performed as well.  This is a painless test measuring whether the intestine is able to relax normally - remember, in Hirschsprung's disease, the absence of nerves prevents the intestine from relaxing normally.

If a child has Hirschsprung's disease, some form of surgery is usually required to eliminate the problems with constipation.