Journal of Pediatric Surgery
Volume 30, Issue 10 , Pages 1481-1484, October 1995

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

  • Saori Nakahara

      Affiliations

    • Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan.
    • the Department of Pediatrics, Toshima Metropolitan Hospital, Tokyo, Japan.
    • ,
  • Kinji Yokomori

      Affiliations

    • Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan.
    • the Department of Pediatrics, Toshima Metropolitan Hospital, Tokyo, Japan.
    • ,
  • Kenichi Tamura

      Affiliations

    • Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan.
    • the Department of Pediatrics, Toshima Metropolitan Hospital, Tokyo, Japan.
    • ,
  • Kikuko Oku

      Affiliations

    • Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan.
    • the Department of Pediatrics, Toshima Metropolitan Hospital, Tokyo, Japan.
    • ,
  • Yoshiaki Tsuchida

      Affiliations

    • Corresponding Author InformationAddress reprint requests to Yoshiaki Tsuchida, MD, Department of Pediatric Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113, Japan.
    • Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan.
    • the Department of Pediatrics, Toshima Metropolitan Hospital, Tokyo, Japan.

Abstract 

The authors report a case of the rare concurrence 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.

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PII: 0022-3468(95)90413-1

Journal of Pediatric Surgery
Volume 30, Issue 10 , Pages 1481-1484, October 1995

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