Hirschsprung’s Disease (HD)
is a congenital disorder of the intestines. It is caused when some of the nerve cells that are normally present in the wall of the intestine do not form properly. These nerve cells, called ganglion cells, help move stool through the intestines in a coordinated fashion called peristalsis. When normal peristalsis cannot occur, stool backs up and causes a partial or complete bowel obstruction. The major symptom is generally severe constipation; sometimes this obstruction may lead to a life-threatening infection (entercolitis) and possible perforation (a hole) in the bowel. The portion of the intestines that is affected can vary from a very short segment in the rectum to the entire colon and part of the small intestines.
Children with HD have severe constipation. They may have other symptoms including, vomiting, explosive foul-smelling diarrhea, poor appetite, poor growth, fever, and abdominal distention.
All Children with HD will require surgery to remove the portion of the rectum/colon that does not have ganglion cells. Some children will require a colostomy for a few months before their pull through surgery (corrective operation); others will have a pull through without a colostomy. Your pediatric surgeon will decide which procedure is best for your child.
Children with HD may develop enterocolitis (infection of the intestines). This is serious and could be life threatening. This can happen if stool and bacteria back up in the colon.
Children with enterocolitis may have:
- swollen belly
- bleeding from the rectum
If your child has these signs, call your child’s doctor immediately. The doctor will want your child to receive rectal irrigations
to rinse stool and bacteria out of the colon. The surgeon may admit your child to the hospital for IV fluids and antibiotics. Enterocolitis may also occur for several years after surgery.
Children with HD will be followed closely throughout childhood and adolescence, with some being followed into adulthood. Many children will have some degree of bowel dysfunction. At the Colorectal Center at Primary Children’s Medical Center we address ongoing problems and coordinate care between specialists to manage your child’s needs. We provide a Bowel Management Program, where we work closely with you and your child to assess function and provide a way for your child to stay clean of stool and prevent accidents.