Hirschsprung’s disease

Alternative Names

Congenital megacolon

Definition of Hirschsprung’s disease

Hirschsprung’s disease is a blockage of the large intestine due to improper muscle movement in the bowel. It is a congenital condition, which means it is present from birth.

Causes, incidence, and risk factors

Muscle contractions in the gut help digested materials move through the intestine. This is called peristalsis. Nerves in between the muscle layers trigger the contractions.

Symptoms

Symptoms that may be present in newborns and infants include:

Signs and tests

Milder cases may not be diagnosed until a later age.

Treatment

Before surgery, a procedure called serial rectal irrigation helps relieve pressure in (decompress) the bowel.

Expectations (prognosis)

Symptoms improve or go away in most children after surgery. A small number of children may have constipation or problems controlling stools (fecal incontinence). Children who get treated early or who have a shorter segment of bowel involved have a better outcome.

Review

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 11/2/2009

ADAM Medical Encyclopedia

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