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Treatments and drugs
By Mayo Clinic staffSurgery is the only proven, effective treatment for Hirschsprung's disease. The procedure is called pull-through surgery and involves removing the section of the colon that has no ganglia cells, then connecting the remaining healthy end of the colon to the rectum.
Sometimes pull-through surgery is done in one step immediately after diagnosis. Other times, a doctor may choose to complete the process in two steps. First, the doctor will remove the abnormal portion of the colon without ganglia cells, and perform an ostomy. This involves creating a small hole (stoma) in the child's abdomen and connecting the top, healthy portion of the colon to the stoma. Stool then leaves the body through the stoma into a bag that attaches to it, allowing the lower part of the colon to heal. A stoma bag must be emptied several times a day. Ostomy may include:
- Ileostomy. In ileostomy, the doctor removes the entire colon and connects the small intestine to the stoma.
- Colostomy. In colostomy, the doctor leaves part of the large intestine and attaches this to the stoma.
Later, after allowing time for the child to recover from the first surgery, the doctor will close up the stoma and perform a second surgery to connect the healthy portion of the colon to the rectum.
Complications of surgery
After surgery, most children pass stool normally. Some may experience diarrhea initially, but after some time stool will become more solid. Toilet training may take longer because some children have difficulty coordinating the muscles used to pass stool. This improves with time in most children. Constipation may continue in some children, although laxatives should help. Eating high-fiber foods also can help with diarrhea and constipation.
A child is also at risk of developing enterocolitis in his or her colon or small intestine after surgery. Be aware of signs and symptoms of enterocolitis, and call the doctor immediately if any of these occur:
- Fever
- Swollen abdomen
- Vomiting
- Diarrhea
- Bleeding from the rectum
- What I need to know about Hirschsprung's disease. National Digestive Diseases Information Clearinghouse. http://digestive.niddk.nih.gov/ddiseases/pubs/hirschsprungs_ez/. Accessed Sept. 12, 2008.
- Hirschsprung's disease. American Academy of Pediatrics. http://familydoctor.org/online/famdocen/home/children/parents/special/birth/906.htm. Accessed Sept. 12, 2008.
- Wesson, DE. Congenital ananglionic megacolon (Hirschsprung disease). http://www.uptodate.com/home/index.html. Accessed Sept. 12, 2008.
- Hirschsprung's disease. American Pediatric Surgical Association. http://www.eapsa.org/parents/resources/hirschsprungs.cfm. Accessed Sept. 12, 2008.
- Hirschsprung's disease. Family Practice Notebook. http://www.fpnotebook.com/GI/Neuro/HrschsprngsDs.htm. Accessed Sept. 12, 2008.
- Endom E. Emergency complications of Hirschsprung disease. http://www.uptodate.com/home/index.html. Accessed Sept. 12, 2008.
- Picco M [expert opinion]. Mayo Clinic, Rochester, Minn. Sept. 17, 2008.
