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.
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