Anorectal manometry. In this test, a small tube is inserted into the rectum to measure how well the muscles in the anus are working. If the muscles don’t relax, it can sometimes be a sign of Hirschsprung’s disease.
How is it treated?
Children with Hirschsprung’s disease need surgery to remove the diseased part of the large intestine. Surgery is often done within the first days or month of life, soon after the disease is found. Treatment may involve one or two surgeries:
The first surgery removes the damaged part of the intestine and may create a colostomy or ileostomy so that the intestine can heal. (With an ostomy, stool leaves the body through an opening in the belly and collects in a bag.)
If your baby gets an ostomy during the first surgery (not all babies do), a second surgery will be done to close the ostomy. This will allow stool to pass through the body normally again.
Most babies are in the hospital from a couple of days up to 1 week. Being involved in your baby’s care while he or she is in the hospital may help you feel more comfortable when you take your baby home. Talk with the doctor about how to feed and care for your baby at home, and make sure you know what problems to watch for. It’s normal to feel nervous, but don’t be afraid to hold and handle your baby.
Some children have long-term (chronic) problems with stomachaches and bowel problems after surgery. But most of the time, these problems aren’t severe. Depending on the problem, there are a number of treatment options. These include medicine, biofeedback, cognitive-behavioral therapy, and more surgery.
In a few cases, emergency surgery may be needed if a dangerous problem such as serious swelling of the small and large intestines (enterocolitis) occurs.
Vanderhoof JA, Young RJ (2006). Hirschsprung disease. In FD Burg et al., eds., Current Pediatric Therapy, 18th ed., pp. 529â€“532. Philadelphia: Saunders Elsevier.
Other Works Consulted
Constipation Guideline Committee (2006). Evaluation and treatment of constipation in infants and children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition, 43(3), pp. e1â€“e13. Also available online: http://www.naspghan.org/wmspage.cfm?parm1=295.
Fiorino KN, et al. (2011). Motility disorders and Hirschsprung disease. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 19th ed., pp. 1283â€“1287. Philadelphia: Saunders.
Kahn E, Daum F (2010). Enteric nervous system section of Anatomy, histology, embryology, and developmental anomalies of the small and large intestine. In M Feldman et al., eds., Sleisenger and Fordtran’s Gastrointestinal and Liver Disease, 9th ed., vol. 2, pp. 1636â€“1641. Philadelphia: Saunders.
Sood MR, Calkins CM (2011). Motor disorders of the stomach, small bowel, and colon. In CD Rudolph et al., eds., Rudolph’s Pediatrics, 22nd ed., pp. 1433â€“1437. New York: McGraw-Hill.
ByHealthwise StaffPrimary Medical ReviewerJohn Pope, MD, MPH – PediatricsKathleen Romito, MD – Family MedicineBrad W. Warner, MD – Pediatric Surgery, Critical Care Medicine