Hirschsprung's disease

Hirschsprung disease (HD) is a disorder of the gut which is caused by the failure of the neural crest cells (enteric ganglion cells) to migrate completely during fetal development of the intestine. The affected segment of the colon fails to relax, causing an obstruction. In the majority of affected people, the disorder affects the short segment of the distal colon. In rare cases the aganglionosis involves more of the colon. In 5 percent of cases the entire colon is affected. Hirschsprung is also sometimes called congenital aganglionic megacolon. Hirshsprung disease occurs in approximately one in 5000 births. The first report of Hirschsprung disease dates back to 1691, however, the disease is named after Harald Hirschsprung, the Danish...