Hirschsprung's disease - Definition

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Definition


Hirschsprung's disease, also known as congenital intestinal aganglionosis, is a rare genetic disease of the digestive system appearing in childhood. It affects 4 out of every 5 boys. It is due to the lack of migration of certain cells which enables the transmission of information in the colon. This results in significant and chronic constipation when manifested in adults. A surgical operation can restore normal intestinal processes by removing abnormal parts of the intestine. In most cases, Hirschsprung's disease occurs at birth as an absence of the first discharge of baby feces, called the meconium, and intestinal obstruction syndrome. In infants with significant constipation, an x-ray of the abdomen called ASP or an enema give reason to suspect the diagnosis, and a rectal biopsy will confirm it.