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Hirschsprung's disease is a birth defect that affects the nerve cells in the large
intestine. These nerve cells control the muscles that normally push food and
waste through the large intestine.
In babies who have
Hirschsprung's disease, the muscles in the wall of the large intestine don't
relax, which prevents stool from passing. This can lead to trapped stool,
infection, pain and swelling, and bowel problems.
Most of the time,
the disease is found soon after birth. It occurs in about 1 out of every 5,000 newborns and is most common in male babies.1
rare cases, the disease can be life-threatening.
know what causes the disease, but it tends to run in families. It may also be
linked to other medical problems, such as
Down syndrome and
congenital heart disease.
Symptoms can depend on how severe the problem is and how old the child is. They may include:
Hirschsprung's disease can lead to serious and even life-threatening problems if it is not found early. Be sure to take your baby for regular checkups, and talk to your doctor if you have concerns.
children are diagnosed with Hirschsprung's disease during their first year. A
doctor may think that a child has the disease based on the child's symptoms and
the results of a physical exam.
Other tests may be done to
confirm the diagnosis, such as:
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
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,
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)
Health Tools help you make wise health decisions or take action to improve your health.
Learning about Hirschsprung's disease:
Vanderhoof JA, Young RJ (2006). Hirschsprung disease. In FD Burg
et al., eds., Current Pediatric Therapy, 18th ed.,
pp. 529–532. 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. e1–e13. Also available online:
Fiorino KN, et al. (2011). Motility disorders and Hirschsprung disease. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 19th ed., pp. 1283–1287. Philadelphia: Saunders.
Gomez J, Parkman HP (2009). Megacolon section of Gastrointestinal motility and functional disorders. In EG Nabel, ed., ACP Medicine, section 4, chap. 14. Hamilton, ON: BC Decker.
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. 1636–1641. 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. 1433–1437. New York: McGraw-Hill.
Current as of:
June 4, 2014
John Pope, MD - Pediatrics & Brad W. Warner, MD - Pediatric Surgery
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