Congenital pouch colon syndrome: A report of 17 cases

BACKGROUND: Congenital pouch syndrome (CPC) is a rare condition seen in association with anorectal malformation that occurs almost exclusively in northern India. We reviewed cases seen in our institution to study aspects of clinical presentation, diagnosis, embryogenesis and management and raise awareness of this relatively infrequent entity. PATIENTS AND METHODS: From March 2002 to September 2004, 17 neonates/infants (11 males and 6 females) treated for CPC associated with anorectal malformations included 13 with type IV and 4 with type I CPC. Diagnosis was made by a single large air-fluid level on the infantogram occupying more than 50% of the entire abdominal dimension. RESULTS: In all patients, the pouch had fistulous communication with the genitourinary system, and there were other associated anomalies as well. Of 13 patients with pouch colon type IV, 11 neonates underwent laparotomy, ligation of the fistula, excision of the colonic pouch and end colostomy as a stage 1 procedure. Subsequently, these patients underwent definitive surgery, i.e. abdominoperineal posterior sagittal anorectoplasty (AP-PSARP), with or without covering colostomy. Two of 4 patients with type I CPC underwent laparotomy, ligation of the fistula and colorrhaphy as a first-stage operation before AP-PSARP. In our series, 4 patients were diagnosed intraoperatively and were treated in accordance with their operative findings. Post-operatively, there were no major complications except wound infection in some patients. There was one death that was not related to surgery. CONCLUSION: There are variants of the anomaly, but the possibility of CPC needs to be kept in mind as a possible association with anorectal malformations.