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

Journal of Pediatric Surgery
Takehito Oshio

Abstract

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 b...Continue Reading

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Citations

Aug 18, 2010·Pediatric Surgery International·Anne-Marie O'Donnell, Prem Puri
Apr 26, 2011·Pediatric Surgery International·Takashi DoiPrem Puri
Dec 31, 2011·Pediatric Surgery International·B L SkellyA C Dick
Aug 21, 2013·Pediatric Surgery International·Alejandro D Hofmann, Prem Puri
Sep 16, 2015·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Alexander CoeRaj P Kapur
Sep 26, 2019·Pediatric Surgery International·Hiroki Nakamura, Prem Puri
Dec 20, 2020·Cellular and Molecular Gastroenterology and Hepatology·Qi YuLiang Wang
Feb 17, 2021·International Journal of Surgery Case Reports·Yousef El-GoharyRichard J Wood
Jun 9, 2020·Journal of Pediatric Surgery·Raphael N Vuille-Dit-BilleAndrea Bischoff

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