What is Hirschprung’s Disease?

Hirschprung’s Disease in a condition in which the nerve cells that are normally present in the large intestine are absent. When these nerve cells are missing, the intestine cannot move waste along toward the anus normally resulting in severe constipation or obstruction of the intestine.  Commonly children are diagnosed around the time of birth with this condition due to a delay in having a bowel movement, although sometimes children are not identified until an older age due to chronic constipation.

How is Hirshhprung's Disease diagnosed?

In order to diagnosis the disorder, a biopsy of the rectal tissue is performed. The condition can involve varying lengths of large intestine beginning at the rectum. Once the involved intestine is identified, this portion is removed and the uninvolved intestine is then used to surgically create a new rectum for the child.

This operation, referred to as a “pull-through”, can be done as either a single or two-stage operation. The two-stage operation involves performing a colostomy which allows the waste to be diverted to the abdominal wall and emptied into a bag. The pull-through is then done at a second operation after the child has resolved other concurrent issues that may complicate their recovery. In the single-stage procedure, the pull-through is performed without a colostomy and is reserved for selected infants or children felt to be candidates for the more complex procedure.

Such complex surgeries are performed routinely, with excellent outcomes, at Children’s Medical Center Dallas.

Learn more about other Gastro-Intestinal conditions

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