The clinical basis for a simplified classification of anorectal agenesis

Sixty consecutive patients with anorectal agenesis were seen during a ten year period. Among 41 boys and 19 girls, there were 35 supralevator and 25 infralevator abnormalities; associated anomalies were present in 38 patients. The over-all mortality was 23 per cent. A simplified classification of anorectal agenesis, based on therapeutic considerations, is proposed. It consists of four basic groups: male high and low and female high and low. A low lesion is defined as having a visible opening or membrane covered opening in the perineum in the male or a visible opening in the introitus or perineum in the female. These infants are managed with simple dilations of the fistula; definitive perineal anoplasty, if necessary, is deferred until the child is six months of age or older. All other lesions are defined as high lesions and are treated in the newborn period with a colostomy. Sacroperineal or sacroabdominoperineal rectoplasty is deferred until the infant is six to 12 months of age. The use of this classification as well as the judgments it implies in the timing of operative procedures will avoid dangerous perineal dissections in the newborn.