Congenital pouch colon associated to a cloaca malformation in a syndromic newborn: A case report

Introduction: Congenital pouch colon (CPC) is a rare intestinal anomaly usually associated with an anorectal malformation (ARM). Because of its low incidence, there are no guidelines on the correct treatment. We report the step-by-step management and challenging surgery of a newborn with CPC and cloaca to ensure the patient's best quality of life possible. Case presentation: We describe a case of type II CPC associated with a complex cloaca with a retrovesical didelphus uterus in a 1530g preterm syndromic female. A preoperative cystoscopy identified a urogenital sinus with an anterior bladder and a posterior vagina. The abdominal exploration done on day 1 showed a dilated CPC with the cecum ending in a large structure extending toward the bladder, and a proximal diverting ileostomy was created. Eight months later, the CPC was mobilized, and the common wall of the bladder and the pouch colon was excised. The 8-cm colonic pouch was tabularized by a triangle resection resulting in a colonic tube used to create a colostomy. Because of the syndromic nature of the patient, the consensus has been not to proceed with the urogenital reconstruction and the ileo-anal pull-through. Conclusion: CPC malformations should be suspected in the case of a single perineal orifice. There is no consensus about the best surgical approach to CPC. The operative reconstruction must be tailored to each patient in order to ensure the best possible quality of life.