Please be patient! It may take up to ONE minute to load all the Engines.
Problems? Please contact our support.
Alternative namesCongenital megacolon
DefinitionHirschsprung's disease is an obstruction of the large intestine caused by inadequate motility (muscular movement of the bowel) that occurs as a congenital (present at birth) condition.
Causes, incidence, and risk factors
Digested matter normally passes through the intestine as a result of the rhythmic contraction of the muscles that line the gut (peristalsis). These muscles are stimulated to contract by nerve bundles, called ganglia, located between the muscle layers.
In Hirschsprung's disease, the ganglia are missing, usually from only a few centimeters of bowel, but sometimes from long segments of bowel. Segments in which there is no peristalsis will not pass any digested matter beyond that point. The end result is that the bowel cannot push material through and is effectively obstructed.
Milder cases may not be diagnosed until a later age. In older children, the disease may be characterized by chronic constipation , abdominal distention, and decreased growth rate.
Signs and tests
During a physical examination, the doctor may be able to feel loops of bowel in the distended abdomen . A rectal examination may detect a loss of muscle tone in the rectal muscles.
Tests used in the diagnosis of Hirschsprung's disease include:
Most cases require surgery. A temporary colostomy (an opening from the bowel to the abdominal wall) is performed as soon as possible to prevent complications associated with bowel obstruction .
Removal of the affected bowel segment and reconnection of the colon is usually performed when the infant is 6 months or older. Antibiotics are given if a hole in the bowel (perforation) or enterocolitis has occurred.
Symptoms are eliminated in 90% of children after surgical treatment. A better outcome is associated with early treatment, and shorter bowel segment involvement.
Calling your health care provider
Call your child's health care provider if symptoms suggestive of Hirschsprung's disease develop.
Update Date: 1/12/2003Andrew J. Muir, M.D., M.H.S., Division of Gastroenterology, Duke University Medical Center, Durham, NC. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT