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Hirschsprung's disease associated with Ondine's curse
R Raveendra Varma1, P K Narayanankutty, K Rajagopalan
1Mother Hospital, Trissur, Kerala, India. rrvarma@bigfoot.com
Indian Journal of Pediatrics
|December 31, 2002
Abstract:
This is a case report of a very rare combination of congenital aganglionosis of the intestine (Hirschsprung's disease) and congenital central hypoventilation syndrome-CCHS- (Ondine's curse). The combination is also called Haddad syndrome. In about 50% of the cases of CCHS there is associated Hirschsprung's disease. There are only about 48 cases reported worldwide. The disease has a very high mortality especially in the settings of a developing country.