Hirschsprung Disease (Aganglionic Megacolon)
What is Hirschsprung Disease?
Hirschsprung Disease is a rare congenital (present at birth) abnormality that results in obstruction because the intestines do not work normally. It is most often found in males. It is commonly found in Down Syndrome children. It can be life-threatening or a chronic disorder. The inadequate motility is a result of an aganglionic (without nerve tissue) section of the intestines resulting in megacolon (dilated section of colon).
What are the symptoms of Hirschsprung Disease?
In a newborn, the chief signs and symptoms are failure to pass a meconium stool within 24-48 hours after birth, reluctance to eat, bile-stained (green) vomiting, and abdominal distension. During infancy the child has difficulty gaining weight , constipation, abdominal distension, episodes of diarrhea and vomiting. Explosive watery diarrhea, fever and exhaustion are signs of enterocolitis (inflammation of the colon) and are considered serious and life-threatening. If these symptoms occur, notify your child's doctor immediately. In older children, symptoms become chronic and include constipation, passage of ribbon-like, foul-smelling stools, abdominal distension and visible peristalsis (wave-like movement of the intestines). The older child is usually poorly nourished and anemic.
How is Hirschsprung Disease diagnosed and treated?
A rectal biopsy is done to diagnose Hirschsprung Disease. Treatment is removal of the aganglionic portion of the colon. Surgical correction is a 1 to 3 stage process. In some cases, a temporary colostomy (an artificial opening from the colon to the skin) is made with the part of the bowel with normal nerve transmission. This colostomy allows the bowel some rest in order to return to its normal function. It also allows the child time for weight gain before the more complicated repair takes place in the next surgery. In the final correction, the pediatric surgery team pulls the working part of the colon down and attaches it to a point near the anus. The colostomy may be closed at this time. The surgery team then waits for the passage of normal stool. If you have any questions, please do not hesitate to ask your nurse or any member of the pediatric surgery team.
Following surgery some children may have problems with persisting constipation. Others can have episodes of enterocolitis as well. Your child will be followed by your pediatric surgeon for these problems.
For additional information on Hirschsprung Disease visit the APSA Family and Parent Resource Center's page on Hirschsprung Disease here »
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This information, although based on a thorough knowledge and careful review of current medical literature, is the opinion of doctors at The University of Texas Medical School and is presented to inform you about surgical conditions. It is not meant to contradict any information you may receive from your personal physician and should not be used to make decisions about surgical treatment. If you have any questions about the information above or your child's care, please contact our doctors.