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Hirschsprung's disease associated with Ondine's curse: a special subgroup?
S Nakahara1, K Yokomori, K Tamura
1Department of Pediatric Surgery, University of Tokyo, Japan.
Journal of Pediatric Surgery
|October 1, 1995
Abstract:
The authors report a case of the rare occurrence of congenital central hypoventilation syndrome (Ondine's curse) and long segmental colonic aganglionosis (Hirschsprung's disease). A review of 24 reported cases showed that the proportion of females having this concurrence is higher than for ordinary Hirschsprung's disease. It also appears that the aganglionic segment is much longer in these cases than in ordinary Hirschsprung's disease.