IMPERFORATE ANUS (ANAL AGENESIS) WITH RECTAL AND SIGMOID ATRESIAS IN A NEWBORN

A male baby was born to a healthy mother after an uncomplicated 39-wee k pregnancy. He had finger and toe syndactyly, right hemidiaphragm eve ntration, and a high imperforate anus (anal agenesis). The kidneys and chromosomes were normal. A right transverse colostomy was done and 5 months later he underwent a posterior sagittal anorectoplasty (Pena pr ocedure). An intermediate imperforate anus with no urethral fistula an d a rectal atresia 2 cm proximal to the distal rectal pouch were found ; the distal rectal pouch was resected and the standard Pena procedure completed. Dilatations were initiated 2 weeks postoperatively and con tinued until the colostomy was closed. Prior to closure of the colosto my, a distal colon loopogram demonstrated an obstruction in the sigmoi d; constrast introduced via the rectum outlined the same obstruction s eparated by 2 cm. A low sigmoid atresia was then repaired. Three weeks later, a contrast loopogram showed a narrow but intact rectosigmoid a nastomois, and the colostomy was closed. The anorectal dilatations wer e discontinued and he remains well at 7 years of age.