Decision making in pediatric colorectal surgery

Most cases of Hirschsprung disease go extremely well, but when patients do not do well after pull-through, it is necessary to determine the cause of the problem.

The presented case shows a 2-year-old with Hirschsprung disease who had a primary pull-through (no colostomy) and is now having obstructive symptoms and recurrent enterocolitis that resolves with irrigations.

The contrast study shows narrowing of the distal colon and very dilated proximal colon, with a change of size in the left colon that could represent a twist or stricture.

A lateral contrast study is valuable for detecting a retained Soave cuff because it can show extra space in the presacral location.

Retained aganglionosis does not explain the anatomic finding of distal narrowing with proximal dilatation seen on contrast study.

A stricture in the location shown does not make sense in a patient who never had a colostomy.

The cutoff seen on contrast study that never goes away on any image indicates the pull-through was twisted as it was brought through the pelvis.

One approach to avoiding a twist is to never do pure transanal Hirschsprung pull-through, but always do laparoscopic dissection from above, then perform laparoscopy again after pull-through to ensure the mesentery is posterior.

When performing transanal-only pull-through, placing a stitch of different color at 12 o'clock or 6 o'clock as soon as the rectum is detached serves as a marker for original orientation.

Despite placing midline orientation stitches, it is still possible to create a 360-degree twist.

At the end of the procedure, placing a Hegar dilator into the colon to see if it passes easily can detect a twist.

Even when performing laparoscopic pull-through, a twist can still occur, but laparoscopic view allows for double-checking the pull-through.

Not all twisted pull-throughs show proximal dilatation; partial twists (180 degrees rather than 360 degrees) may not cause dilatation.

To detect a twist on rectal examination in a post-pull-through Hirschsprung patient, perform rectal exam while palpating with the other hand on the abdomen; if you can feel your finger inside the abdomen there is no twist, but if there is tissue between your finger and hand, this suggests a twist.

In a 2-year-old child (approximately 14-15 kg), an examiner's index finger may only be 7-8 cm long and may not reach the twist, making bimanual palpation technique unreliable in small children.

If finger length is insufficient for bimanual examination, rigid or flexible sigmoidoscopy should be used to ensure a twist is not missed.

When there is size discrepancy between proximal colon and anal canal, if the discrepancy is not too large, it is possible to plicate the proximal colon and adapt it to the anal canal, and long-term the discrepancy will decrease.

If the colon is too wide, one option is to go higher up where the colon is thinner rather than at the maximally dilated segment.

Plication creates a difficult anastomosis to perform.

Rather than formal plication in one location, a harmonic anastomosis without too much discrepancy can be performed.

For connecting a large circle to a small circle, place stitches at 12, 6, 3, and 9 o'clock positions first, put all four on mosquito clamps, hold the mosquitoes apart to straighten the size discrepancy, then place a stitch in the middle and continue dividing in half each time.

The circle-to-circle mosquito technique can also be used in the abdomen for intestinal atresia with dilated proximal and non-dilated distal bowel.

Tapering or plicating in Hirschsprung disease is not recommended because it creates opportunity for stasis.