Hirschsprung's disease associated with Ondine's curse: a special subgroup?

J Pediatr Surg. 1995 Oct;30(10):1481-4. doi: 10.1016/0022-3468(95)90413-1.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Female
  • Hirschsprung Disease / complications*
  • Humans
  • Infant, Newborn
  • Male
  • Sleep Apnea Syndromes / complications*
  • Sleep Apnea Syndromes / physiopathology