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J Pediatr Surg. 2008 Jan;43(1):222-6. doi: 10.1016/j.jpedsurg.2007.09.005.

Imperforate anus, malrotation, and Hirschsprung's disease with double zonal aganglionosis: an extremely rare combination.

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Department of Surgery, National Kagawa Children's Hospital, Zentsuji-Town, Zentsuji-City, Kagawa-ken 765-8501, Japan.


The author describes an extremely rare case of coexistence with imperforate anus, malrotation, and double zonal aganglionosis. A colostomy was performed on a 2-day-old male infant at the proximal sigmoid colon, because the finding of an invertgram revealed an intermediate type of imperforate anus. At the age of 1 month, a distal colostogram showed the low type without fistula. An upper gastrointestinal series revealed malrotation. At 4 months of age, Ladd's procedure and an anoplasty were done. The function of the colostomy was not good. At laparotomy, a narrowing terminal ileum was removed for being a suspected intestinal obstruction. Histologic findings of the removed ileum revealed aganglionosis. A rectal suction biopsy showed the positive finding of acetyl cholinesterase staining. Therefore, the patient was diagnosed with extensive aganglionosis. At 9 months of age, a 1-stage ileoendorectal pull-through with a right colon onlay patch was performed. Histologically, a skipped ganglionic bowel segment at the right colon, a double zonal aganglionosis, was found among the extensive aganglionosis. Although the ganglionic right colon was used for the colon patch, which was placed for antiperistaltic movement, postoperatively the bowel function was excellent. The author herein describes an extremely rare case of coexistence of imperforate anus, intestinal malrotation, and double zonal Hirschsprung's disease. A case with this association, to the author's knowledge, has not been reported previously in the literature.

[Indexed for MEDLINE]

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