Hirschsprung Disease: Update Course 2015

For a newborn with Hirschsprung disease and a transition zone at the descending colon level, laparoscopic biopsy and mobilization is the preferred approach among polled experts.

For the average pediatric surgeon performing primary transanal pull-through without biopsy, there is approximately a 1 in 10 to 1 in 15 chance of encountering disease higher than expected or total colonic aganglionosis during their career.

Surgeons performing transanal pull-through without prior biopsy should have a predetermined exit strategy for managing unexpectedly proximal disease.

The safest approach to Hirschsprung surgery involves some form of biopsy (laparoscopic or full-thickness through umbilicus) prior to definitive pull-through.

Leveling colostomy may be the safest approach when institutional pathology expertise in reading Hirschsprung biopsies is limited or unavailable.

Leveling colostomy represents a three-stage procedure for Hirschsprung disease management.

Pure transanal pull-through may not be less invasive than laparoscopic approach, as prolonged torquing in the anal canal can be more traumatic than three small abdominal incisions.

Transanal dissection attempting to reach high past the pelvic reflection involves significant pulling and stretching of the sphincters.

Prolonged transanal dissection (approaching 4 hours) should prompt consideration of converting to laparoscopic approach to avoid excessive sphincter stretching.

Laparoscopic mobilization with 3-millimeter ports can be completed in approximately 45 minutes for appropriate cases.

Standard rectosigmoid Hirschsprung disease (6-10 centimeters proximal) can be completed transanally in approximately 2 hours when the level is known from a good contrast study.

Complications after Hirschsprung surgery are divided into two categories: obstructive symptoms (including enterocolitis, failure to thrive, abdominal distention) and soiling issues.

Soiling after Hirschsprung surgery is further classified into true incontinence and pseudo-incontinence.

Obstructive symptoms after Hirschsprung surgery require differentiation between anatomic problems and pathologic problems.

True fecal incontinence after Hirschsprung surgery can result from injury to the sphincter or injury to the dentate line.

Pseudo-incontinence after Hirschsprung surgery may be caused by underlying constipation.

Workup for post-Hirschsprung complications includes water-soluble contrast enema and exam under anesthesia evaluating dentate line position, stricture, stretched sphincter, and anatomic twists.