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Hirschsprung's disease - Treatment

Alternative Names

Congenital megacolon

Treatment:

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

The abnormal section of colon must be removed with surgery. Most commonly, the rectum and abnormal part of the colon are removed. The healthy part of the colon is then pulled down and attached to the anus.

Sometimes this can be done in one operation. However, it is often done in two parts. A colostomy is performed first, and another procedure is performed later in the child's first year of life.

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.

Complications:

  • Inflammation and infection of the intestines (enterocolitis) may occur before surgery, and sometimes during the first 1-2 years afterwards. Symptoms are severe, including swelling of the abdomen, foul-smelling watery diarrhea, lethargy, and poor feeding.
  • Perforation or rupture of the intestine
  • Short bowel syndrome, a condition that can lead to malnourishment and dehydration

Calling your health care provider:

Call your child's health care provider if:

  • Your child develops symptoms of Hirschsprung's disease
  • Your child has abdominal pain or other new symptoms after being treated for this condition
  • Reviewed last on: 11/2/2009
  • 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.

References

Kessmann J. Hirschsprung's Disease: Diagnosis and Management. Am Fam Phys. 2006;74:1319-1322.

Wyllie R. Motility disorders and Hirschsprung disease. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 329.

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