What the Heck is Hirschsprung Disease?

Dr. Marc Levitt is Surgical Director for the Center for Colorectal and Pelvic Reconstruction at Nationwide Children's Hospital. He contributed this article to Live Science's Expert Voices: Op-Ed & Insights.

One in 5,000 babies is born with Hirschsprung disease. It is a congenital condition in which nerve cells in the colon don't develop properly so the child is born without key nerve cells — nerves responsible for pushing food through the intestines so that it can be digested and passed out of the body.

When the nerves are missing, stool gets "backed up" in the intestines. Bacteria that are normally present begin to overgrow, causing an infection, which leads to severe diarrhea and dehydration. The infection can be quite serious, even deadly. [7 Baby Myths Debunked ]

Hirschsprung disease develops while a baby is still growing inside the womb . More boys than girls are affected and children with Down syndrome have a higher incidence. The condition may be hereditary — there is a slightly increased chance that a couple will have a child with Hirschsprung disease if one of the parents has the condition, or if they have already had one child with the condition.

Since people with Hirschsprung disease are born with it, they are generally diagnosed when they are infants. Less severe cases are sometimes diagnosed when a child is older.

Signs and symptoms of Hirschsprung disease can vary depending on the severity of the condition. Often, the most obvious sign of Hirschsprung disease is a newborn's failure to have a bowel movement within 48 hours after his or her birth.

This can also be a sign of other medical conditions including, but not limited to, meconium ileus, small left colon syndrome or hypothyroidism. Therefore, tests and procedures are initiated to rule out conditions during the process of evaluating the patient and identifying the underlying problem.

Other signs and symptoms in newborns may include:

• Bloating of the abdomen
• Vomiting (vomit may appear green or brown in color)
• Constipation or gas
• Diarrhea
• Fever

Children with Hirschspring disease will always need surgery to remove the non-functional part of their intestine, and to restore the ability to move stool through the digestive tract and out of the body. Nowadays, in many cases this complicated operation can be done all through the anus with no incisions at all.

With proper treatment and surgical intervention, the long-term outcomes for children with Hirschsprung disease are excellent. Most will go on to recover normal bowel control and function, allowing them to live happy, healthy lives.

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