The embryology and management of congenital pouch colon associated with anorectal agenesis.
Forty-one infants with a pouch colon malformation accompanied by a high anorectal anomaly were treated between January 1986 and December 1990. The 41 cases constituted 9% of all anorectal malformations and 15.2% of high defects managed during this period. There were 32 boys and nine girls; three of the girls had an associated cloaca. Many of the babies presented in poor condition, with gross abdominal distension caused by the distended colonic pouch. The typical radiological feature was an enormously distended colonic shadow occupying more than 50% of the width of the abdomen. At the time of surgery, the patients were classified into 4 subgroups based on the length of the normal colon. All but three infants had a high wide fistula, with the genitourinary tract consisting of a colovesical fistula in males and a colovaginal or colocloacal fistula in females. Frequent associated malformations included duplication of the appendix and vesicoureteric reflux. The operations performed initially were a window colostomy of the pouch with or without division-ligation of the fistula, end-colostomy after fistula ligation, or subtotal pouch excision with tubularization of the remaining colon and end-colostomy. Thirteen of the 41 patients have undergone a definitive pull-through operation using the posterior sagittal approach, including two children in whom one-stage reconstruction of a cloaca was performed. Standardized management of this complex anomaly is proposed for the initial operation and for definitive reconstruction.