Hirschsprung's disease natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

If left untreated, Hirschsprung disease can lead to enterocolitis and death. After proper surgical intervention, mortality drops significantly. Common complications include enterocolitis, intestinal perforation and short bowel syndrome.

Natural history

If left untreated, Hirschsprung disease can lead to enterocolitis and death. After proper surgical intervention, mortality drops significantly.

Complications

• Inflammation and infection of the intestines (enterocolitis).
• Intestinal perforation.
• Short bowel syndrome, a condition that can lead to malnourishment and dehydration.

Possible complications of surgery include

• Wound infection
• Anastomotic leakage
• Intestinal obstruction
• Pelvic abscess
• Chronic obstructive symptoms
• Chronic constipation or diarrhea

Prognosis

• Hirschsprung disease can lead to mortality in up to 80% of the cases.
• After proper surgical intervention, mortality drops to 30 %.