My five-day-old niece has been diagnosed with Hirschphrung's disease and has to undergo an operation in the coming weeks. Can you please explain to me a little bit more about this condition and what is the long-term prognosis for her?
Hirschprung’s disease is a congenital disorder of the colon. The condition involves a segment of the colon and presents during infancy as a form of intestinal obstruction. The essential feature of the condition is a paralysis of a segment of the gut, which is due to the absence of nerve ganglions in the lining of that particular gut segment. The severity of symptoms depends on the length of colon that is paralysed. The condition is treated surgically and this involves the removal of the paralysed segment of colon. Surgery is curative and the majority of affected children do very well. Hirschprung’s disease solely affects the colon and there are no associated abnormalities.