Congenital pouch colon syndrome in a Saudi Arabian neonate

Congenital pouch colon syndrome is partial or complete replacement of the colon by a pouch-like structure and anorectal malformation. Commonly reported from northern India, we believe this is the first report of congenital pouch colon syndrome in a Saudi Arabian neonate. He was referred with an imperforate anus and diagnosed as having a high anorectal malformation. The patient underwent a sigmoid colostomy. Continued abdominal distension after the colostomy prompted sonography and computed tomography, which showed an air- and fluid-containing cystic structure in the abdomen. Exploration revealed a thick-walled cystic structure in continuity with the distal colostomy and with a blood supply mainly from the superior mesenteric artery. The ureters were dilated and tortuous and the pouch opened into the posterior vesical wall. The pouch was resected and an end colostomy fashioned. The etiopathogenesis classification and management of the congenital pouch colon syndrome are discussed.