January 2007

Leading-Edge Surgery Corrects Anorectal Malformation

Cincinnati Children's Colorectal Center is the only one of its kind in the world.
Watch a PSARP operation, repair of a child with imperforate anus and a rectourethral fistula.

Each year approximately 600 infants are born in the United States with an anorectal malformation (imperforate anus), a common congenital anomaly present in one of every 5,000 births worldwide. Usually diagnosed by pediatricians in the newborn nursery, anorectal malformations are now detected prenatally as well.

Newborns diagnosed with anorectal malformations usually require one or more operations to correct them. The first procedure usually is a colostomy that allows growth to occur and minimizes the risk of infection before the second procedure.

A complex reconstructive surgery called posterior sagittal anorectoplasty (PSARP) follows the newborn temporary colostomy, creating a connection between the rectum and the newly created anal opening. Pioneered in 1980 by pediatric surgeon Alberto Peña, MD, this procedure, also called the Peña procedure or the pull-through procedure, is performed today at Cincinnati Children's by Dr. Peña and pediatric surgeon Marc Levitt, MD, Dr. Peña's colleague for the past 15 years. The Cincinnati Children's team, part of the multidisciplinary Colorectal Center for Children, performs more than 200 complex colorectal procedures each year – more than any other center in the world.

PSARP usually is appropriate for patients older than 1 month, as long as the infant is growing and developing normally. In a recent live-demonstration surgery during the 42nd Surgical Treatment of Anorectal Malformations Course held at Cincinnati Children's, Dr. Peña operated on an infant boy from Greece. The procedure was performed with the child positioned face down, with a Foley catheter inserted in the bladder. Dr. Peña made a midline (mid-buttock) posterior sagittal incision opening the buttocks and exposing the intricate anatomy below.

The sphincter mechanism was divided in a midline incision, preserving the nerve fibers, decreasing the amount of postoperative pain, and avoiding injuries to adjacent structures like the vas deferens, seminal vesicles, urethra and ectopic ureters.

The rectum was then meticulously separated from the genitourinary tract, dissected and freed enough to reach its normal site without tension. The fistulous connection with the urinary tract was closed. With the use of an electrical muscle stimulator, the limits of the sphincter mechanism were determined and the rectum was placed in its optimal location to achieve the best functional results.

Although it was a challenging case because the child's rectum was quite high, Dr. Peña was able to bring it down through the mid-buttock incision, avoiding any incision on the abdomen. This significantly minimized pain and allowed the baby to leave the hospital after 48 hours.

Seventy-five percent of patients with anorectal malformations achieve successful fecal continence. In 25 percent of patients the muscles are not formed well enough to be continent, but a special bowel management program exists at the center so they are clean and dry and in normal underwear.