Pediatric Colorectal Contraversies Part I: Pediatric Colorectal Contraversies...

Low anorectal malformations (bucket-handle and perineal fistula) are typically managed with local perineal procedures (anoplasty) at birth in stable patients.

When a perineal fistula is visible, the rectum is usually located low, but anatomic variants exist where following the narrow track leads to finding the rectum located much higher than expected.

When an unexpected high rectal location is encountered during attempted perineal repair, the surgeon must use clinical judgment to decide whether to continue or convert to colostomy.

All patients with perineal fistulas must have an AP film of the sacrum (not only lateral) to detect sacral defects that indicate presacral masses.

Perineal fistula is the anorectal malformation most commonly associated with presacral masses.

Presacral masses cause a very narrow fibrotic anus that interferes with dilation, and patients subjected to dilations may fail only because the presacral mass diagnosis was missed.

Perineal fistula with presacral mass and sacral defect runs more frequently in families than other anorectal malformations, warranting screening of all family members for sacral defects.

100% of patients with perineal fistula operated without presacral mass have bowel control, but presence of presacral mass and sacral defect changes the prognosis.

In females with anterior fistula and adequately sized anal opening (12 Hegar dilator), observation without surgery is an acceptable approach, with one series following 21 girls (median age 7 years) without surgical intervention.

Surgical indications for female perineal fistula include: hole too small, distal aspect is fistula tissue (not mucosa) that remains stenotic, and malposition outside the sphincter center, which leads to dilated rectosigmoid and severe constipation.

Patients with untreated perineal fistula have good bowel control but may have imperfect control as adults, with problems during loose stools or athletic activity.

Surgical goals for perineal fistula repair are: adequately sized hole, centered within the sphincter, and adequate length perineal body.

Cutback procedure is an operation for bad surgeons or good surgeons working under very difficult circumstances with very sick babies; it is a temporary procedure but patients subjected to it also have bowel control.

The most important message about perineal fistula is that patients will suffer the worst constipation in the spectrum of anorectal malformations, requiring aggressive management with laxatives at doses 2, 3, 5, or 10 times more than standard recommendations.

In the spectrum of anorectal malformations, the lower the malformation, the more severe the constipation; the higher the malformation, the less constipation (with exceptions).

Constipation in perineal fistula patients is lifelong and does not follow standard dosing guidelines from textbooks.

In China, surgeons prefer cutback procedure for male perineal fistula because functional results are good and the procedure is easy to perform.

The sphincter center can be identified visually as an ellipse of red, pink tissue on the perineum.

Since 1948, surgeons have attempted primary repair of anorectal malformations; if lucky enough to find the rectum immediately, successful operation is possible, but this should not be generalized as standard practice.

Cross-table lateral film (replacing the invertogram) is performed by placing the baby in posterior sagittal position, placing the film on the lateral side, with the x-ray beam entering the other side, producing the same image as an invertogram.

If cross-table lateral film shows gas below the coccyx, an experienced, meticulous surgeon can be sure of finding the rectum via posterior sagittal approach and may successfully repair the malformation primarily.

Attempting primary repair without finding the rectum causes serious problems for the baby; such attempts are 'adventures that may become misadventures with serious consequences.'

Adult women with uncorrected perineal fistula may be upset for psychological reasons about having the anal opening very close to the vagina, and there is potential risk of serious rectal injury during vaginal delivery.

For male newborns with flat buttocks and no visible fistula at 24 hours of life with abdominal distension, colostomy is the appropriate first procedure rather than primary repair.