Long-segment Hirschsprung's disease - nerve cells are missing from most or all of the large intestine and could be missing from the last segment of the small intestine.
Short-segment Hirschsprung's disease - nerve cells are missing from the last segment of the large intestine.
Entire large and small intestine - Although rare, in some children the nerve cells are missing.
Symptoms in infants (up to 1 year) can include:
Failure to pass meconium (stool) after birth
Failure to pass first stool within 48 hours of birth
Infrequent, but explosive, stools
Poor feeding
Watery diarrhea
Symptoms in children older than 1 year can include:
Failure to respond to oral constipation medicines
Slow growth
Swelling of belly
Unexplained fever
Hirschsprung’s disease affects five times as many male babies as it does female babies. In a few cases, a dangerous infection called enterocolitis can occur. This infection damages the lining of the intestines, which is a serious issue. In extreme cases, the intestine may also rupture or burst.
In order to diagnose the disorder, a biopsy of the rectal tissue is performed. Learn more about pediatric rectal suction biopsy.
Children suspected of having the disease should get immediate medical treatment, including:
Antibiotic therapy if enterocolitis develops
IV fluids
No food by mouth
Relieving pressure in the intestines and stomach by using enemas
Some cases may require surgery. 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 laparoscopic pull-through surgery can be done as either a single or two-stage operation.
The two-stage operation involves performing a colostomy which allows the stool 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.