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Eur J Pediatr Surg 1983; 38(5): 312-315
DOI: 10.1055/s-2008-1059995
© Georg Thieme Verlag KG Stuttgart · New York

Double Zonal Aganglionosis with a Skipped Oligoganglionic Ascending Colon

Zweifache segmentale Aganglionose mit oligoganglionärem Colon ascendensT.  Taguchi1 , K.  Tanaka1 , K.  Ikeda2 , A.  Hata3
  • 1Department of Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan
  • 2Department of Pediatric Surgery, Faculty of Medicine, Kyushu University, Fukuoka, Japan
  • 3Department of Surgery, Oita Prefectural Hospital, Oita, Japan
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Publication History

Publication Date:
25 March 2008 (online)

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Abstract

We report a case of aganglionosis which involved the rectum, entire colon, appendix, terminal ileum but spared the ascending colon. This case was considered to represent for so-called double zonal aganglionosis. Histologically, the spared ascending colon proved to be an oligoganglionic segment. This may be the first documentation of a skipped oligoganglionic colon in double zonal aganglionosis.

Zusammenfassung

Es wird ein Fall einer Aganglionosis des Rektums, Colon descendens und transversum, der Appendix und des terminalen Ileums, jedoch mit normaler Ganglienzellbesiedelung des Colon ascendens berichtet. Es handelt sich somit um den ersten Fall einer sogenannten doppelten Aganglionose. Histologisch fand sich im Colon ascendens eine Oligoganglionie.

Key words

Hirschsprung's disease - Double zonal aganglionosis - Skipped oligoganglionic segment - Colitis

Schlüsselwörter

Hirschsprungsche Erkrankung - Segmentale Aganglionose - Oligoganglionie - Kolitis