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Treatments and drugs
By Mayo Clinic staff
Surgery
Hirschsprung's disease is treated with surgery to remove the portion of the colon that has no ganglia cells. The remaining portions of the colon and rectum are then joined, so that waste can leave the body normally. Surgery for Hirschsprung's disease is often done in one step immediately after diagnosis.
In children who are very ill, 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 colon and attaches this to the stoma.
After allowing time for the child to recover from the first surgery, the doctor will perform another procedure to close up the stoma and connect the healthy portion of the intestine to the rectum.
Results 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 can 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:
- Bleeding from the rectum
- Diarrhea
- Fever
- Swollen abdomen
- Vomiting
- What I need to know about Hischsprung disease. National Institute for Diabetes and Digestive and Kidney Disease. http://digestive.niddk.nih.gov/ddiseases/pubs/hirschsprungs_ez/. Accessed Oct. 12, 2010.
- Wyllie R. Motility disorders and Hirschsprung disease. In: Kliegman RM. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/208746819-6/0/1608/0.html. Accessed Oct. 12, 2010.
- Kahn E, et al. Anatomy, histology, embryology and development anomalies of the small and large intestine. In: Feldman M, et al. Sleisinger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa.: Saunders; 2010. http://www.mdconsult.com/book/player/linkTo?type=bookHome&isbn=978-1-4160-6189-2&eid=4-u1.0-B978-1-4160-6189-2..X0001-7--TOP&uniq=200844987-3. Accessed Oct. 12, 2010.
- Philichi L. When the going gets tough: Pediatric constipation and encopresis. Gastroenterology Nursing. 2008;31:121.
- Nutrition therapy for constipation. ADA Nutrition Care Manual. http://nutritioncaremanual.org/index.cfm. Accessed Oct. 12, 2010.
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