A developmental defect of the intestinal tract where the muscles of the intestines are unable to function and move food along the digestive tract. This form of Hirschsprung disease is differentiated from the other types by the location of the genetic defect
* Severe constipation * Intestinal blockage * Enlarged colon
In Hirschsprung’s disease, the aganglionic bowel segment contracts without the reciprocal relaxation needed to propel feces forward. In 90% of patients, this aganglionic segment is in the rectosigmoid area, but it occasionally extends to the entire colon and parts of the small intestine. Hirschsprung’s disease is believed to be a familial, congenital defect. It’s up to 5 times more common in males than in females. The disease typically coexists with other congenital anomalies, particularly trisomy 21 and anomalies of the urinary tract such as megaloureter.
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Surgical treatment involves pulling the normal ganglionic segment through to the anus. However, corrective surgery is usually delayed until the infant is about 6 months old so that he’s better able to withstand surgery. Postsurgical management should focus on reestablishing normal fluid and electrolyte balance, preventing bowel distention, and managing complications such as sepsis. Treatment measures include I.V. hydration, nasogastric decompression, and I.V. antibiotics as indicated. Management until the infant is old enough for surgery may consist of daily colonic lavage to empty the bowel. Physiologic saline solution should be used rather than tap water to prevent water intoxication. If total obstruction is present in the neonate, a temporary colostomy or ileostomy is necessary to decompress the colon. Antibiotics are given if the bowel has been perforated or if the infant has enterocolitis.