Hirschsprung Disease Audience Q&A with Dr. Marc Levitt

Botox paralyzes skeletal muscle but clearly has some impact on smooth muscle

Babies have very tight anal sphincter with or without Hirschsprung's disease, and if they hold stool in successfully, normal babies get constipated while Hirschsprung babies get enterocolitis

After a perfectly done operation that preserves 1 centimeter of anal canal without hurting sphincters, high tone will occur in a baby that doesn't know how to relax

Botox is valuable for babies coming back with enterocolitis episodes after ensuring no anatomic or pathologic problem with the pull-through

In the early period up to one year of age, bad behavior by the patient can occur even with a perfectly done pull-through

After one year of age, there may be an anatomic or pathologic problem, and Botox is not very valuable because the underlying problem must be identified

A retained cuff that is too big, not split completely, or rolled up is particularly offensive to the ability of the pull-through to empty

Patients with retained cuff will get better temporarily with Botox but will continue to fail every 2 or 3 months

Nerves greater than 40 microns indicate a transition zone pull-through

Many pathologists are not measuring nerve caliber, and pediatric surgeons should demand that their pathologists do this to avoid doing a pull-through in the transition zone

When anatomic and pathologic issues are completely ruled out (no twist, stricture, cuff, etc.), Botox may be done once or maybe twice and then the patient is done

If a patient over 1 year of age keeps misbehaving, there is likely an anatomic or pathologic problem

Dr. Levitt has never met a patient with Hirschsprung disease that is anatomically perfect (no stricture, cuff, duhamel pouch causing trouble, twist) with normal ganglion cells and nerves less than 40 microns who does not spontaneously empty, except rare patients under one year who have not learned to relax their anal canal

If symptoms persist in an older child, there is an anatomic or pathologic problem that has not yet been identified

Dr. Levitt's Botox technique: 100 units in 10 cc saline, injecting 2.5 cc submucosal into each quadrant with a very small gauge needle

Dr. Levitt will never do internal sphincterotomy because it is permanent Botox and could cause permanent incontinence

Most practitioners use between 60 and 100 units of Botox for Hirschsprung patients

Some practitioners use much less volume (1 mL total with 0.25 mL per quadrant) compared to Dr. Levitt's 10 mL technique

A dilated pull-through segment may be secondary to noncompliance with dilations or bowel regimen, or the surgeon may not have taken out enough bowel at the original surgery

Patients with dilated segment and no other anatomic abnormality who continue to misbehave may be offered redo surgery to remove the dilated segment, though this is exceedingly rare

Almost always there is something causing the dilation, either a cuff or a transition zone segment of bowel

The workup for patients with dilated segments includes contrast study, examination under anesthesia, and rectal biopsy

For redo pull-through, Dr. Levitt performs transanal approach saving the anal canal, delivers bowel into abdomen, then decides whether tapering is needed

A tapered segment will be fairly dysmotile for many months

Dr. Levitt always diverts patients with an ileostomy if tapering or redo is required

Most likely the dilated segment can be removed and healthy segment brought down without tapering

It is critical to ensure removal of any distal obstruction during redo surgery; Dr. Levitt has seen patients redone with Soave cuff left alone, requiring redo of the redo to remove the cuff before improvement