Hirschsprung's disease is treated with surgery to bypass the part of the colon that has no nerve (ganglia) cells. The lining of the diseased part of the colon is stripped away, and normal colon is pulled through the colon from the inside and attached to the anus. This is usually done using minimally invasive (laparoscopic) methods, operating through the anus.
In children who are very ill, surgery may be done in two steps.
First, the abnormal portion of the colon is removed and the top, healthy portion of the colon is connected to a small hole (ostomy) the surgeon creates in the child's abdomen. Stool then leaves the body through the ostomy into a bag that attaches to the end of the intestine that protrudes through the hole in the abdomen. This allows time for the lower part of the colon to heal.
Ostomy procedures include:
- Ileostomy. With an ileostomy, the doctor removes the entire colon. Stool leaves the body through the end of the small intestine.
- Colostomy. With a colostomy, the doctor leaves part of the colon intact. Stool leaves the body through the end of the large intestine.
Later, the doctor closes the ostomy and connects the healthy portion of the intestine to the rectum or anus.
In a small percentage of children, the entire colon and sometimes a part of the small intestine lacks nerves. The usual treatment in this case is to remove the colon and connect remaining healthy bowel to the anus.
Results of surgery
After surgery, most children pass stool normally — although some may experience diarrhea initially.
Toilet training may take longer because children have to learn how to coordinate the muscles used to pass stool. Long term, it's possible to have continued constipation, swollen belly and leaking of stool (soiling).
Children continue to be at risk of developing bowel infection (enterocolitis) after surgery, especially in the first year. Be aware of signs and symptoms of enterocolitis, and call the doctor immediately if any of these occur:
March 28, 2013
- Bleeding from the rectum
- Swollen abdomen
- 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 Feb. 1, 2013.
- Kliegman RM. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa.: Saunders Elsevier; 2011. http://www.mdconsult.com/das/book/body/208746819-6/0/1608/0.html. Accessed Feb. 1, 2013.
- Gunnarsdottir A, et al. Modern treatment of Hirschspring's disease. Scandinavian Journal of Surgery. 2011;100:243.