Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passag … Hirschsprung Disease , also known as congenital aganglionic megacolon, is a rare but serious condition that affects the large intestine (colon) of infants. It is characterized by the absence of nerve cells in certain parts of the colon, leading to problems with bowel movements Hirschsprung 's disease is a birth defect in which nerves are missing from parts of the intestine, causing constipation and other symptoms. Learn about the causes, types, diagnosis, treatment and complications of this genetic disorder. Hirschsprung disease , a neurocristopathy, is the most common cause of neonatal colonic obstruction (15-20%). It is commonly characterized by a short segment of colonic aganglionosis affecting term neonates, especially boys. Rarely, it can present in patients >10 years, termed adult Hirschsprung disease .
