Hirschsprung Disease Rapid Fire: Update Course 2015

When performing primary transanal pull-through without biopsy, approximately 1 in 10 to 1 in 15 cases will have a transition zone higher than expected or total colonic aganglionosis

Leveling colostomy may be the safest approach when pathology support is limited or unavailable, such as on mission trips

Placing three abdominal incisions may be less invasive than torquing in the anal canal during prolonged transanal dissection

Transanal dissection should not involve stretching sphincters for 4 hours; if dissection is taking that long, laparoscopic approach should be used

Laparoscopic approach with three 3-millimeter ports allows mobilization in approximately 45 minutes

Standard rectosigmoid Hirschsprung (6-10 centimeters up) can be completed transanally in approximately 2 hours

Postoperative Hirschsprung complications are categorized into obstructive symptoms (enterocolitis, failure to thrive, abdominal distention) and soiling issues (true incontinence versus pseudo-incontinence)

Obstructive symptoms after Hirschsprung surgery must be evaluated to distinguish anatomic problems from pathologic problems

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

Workup for postoperative Hirschsprung problems includes water-soluble contrast enema and exam under anesthesia looking for dentate line position, stricture, stretched sphincter, and twists

If no anatomic reason is found for postoperative problems, biopsy should be performed

A normal Hirschsprung anus should appear as a normal appearing anus with a normal anal canal, not patulous

Dissection should begin approximately 1 centimeter above the dentate line in Hirschsprung surgery

A 1-centimeter distance above the dentate line in a newborn may become 2.5 to 3 centimeters when the child is 7 years old

If the anastomosis is performed too high, the patient may develop what some call short segment or ultra short segment Hirschsprung disease

Injury to the dentate line can render patients potentially fecally incontinent, which is a devastating injury

The dentate line is defined as the transition from squamous epithelium to columnar epithelium

The top of the anal columns is used as a landmark for the dentate line location

In J-pouch surgery for ulcerative colitis or FAP, the anastomosis is performed right at the top of the columns or slightly lower if polyps are present

The dentate line location in anatomic literature and illustrations is variable, with different sources pointing to different locations within the anal columns

Surgeons may intentionally leave a zone of aganglionosis to avoid fecal incontinence, as residual aganglionosis can be managed with laxatives but incontinence cannot be overcome

The surgical approach should hedge on the side of leaving ultra short segment Hirschsprung disease rather than injuring the anal canal