Decision Making in Colorectal Surgery
With Dr. Mark Levitt & Dr. Alejandra Villanova Sanchez & Dr. Paula Mio & Dr. Augusto Zani
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Older patients with Hirschsprung disease tend to not have serious enterocolitis like babies; they may present with chronic constipation instead
A good rectal biopsy must show ganglion cells or their absence, comment on nerve size, and contain no squamous epithelium (which indicates biopsy taken too low)
Many eosinophils in the presence of ganglion cells on rectal biopsy in a baby can indicate milk protein allergy, which can mimic Hirschsprung disease on X-ray
In Hirschsprung disease, we treat the patient, not the biopsy - a patient doing well with histologic Hirschsprung may not require surgery
Ultra-short Hirschsprung is a confusing term that should be avoided; if ganglion cells are present 1 centimeter proximal to the dentate line, it is not Hirschsprung disease
Patients with ganglion cells within 1 centimeter of the anal canal can have severe constipation and even enterocolitis, but this is internal sphincter achalasia, not Hirschsprung disease
For internal sphincter achalasia (ganglion cells present but sphincter dysfunction), Botox treatment is preferred over myectomy because myectomy can cause permanent sphincter damage
For rectal biopsy in older patients, at least 1 centimeter above the dentate line in clearly columnar epithelium is adequate; 3 centimeters is too far
A flat distal rectum at the pubococcygeal line on contrast enema indicates inadequate pressure, not absence of rectum
The pubococcygeal line represents normal compression of the rectum by pelvic musculature; seeing this on contrast study means the study is inadequate
In perineal fistula cases, adequate pressure contrast enema shows a very deep and reachable rectum, typically within 1-2 centimeters, in almost every case
High anorectal malformation with long fistula is a concept that likely comes from inadequate pressure studies; it is very rare, seen maybe once or twice in hundreds of patients
In male anorectal malformations, a long fistula commonly parallels the urethra; during PSARP, this fistula should be left alone as it will disappear - dissecting it risks urethral injury and spongiosum bleeding
If meconium pearls are visible within the first 24 hours in a patient with covered anus, this indicates low anorectal malformation and primary PSARP can be performed without colostomy
To avoid misplaced anoplasty during PSARP, mark the sphincters before starting the incision using sphincter stimulation, and match that location once open
Using two stitches (one anterior, one posterior margin) on both sides to mark anoplasty location is more reliable than marking pen, which disappears during surgery
Even with perfectly placed anoplasty, fecal incontinence can be the final outcome due to poor sacrum, poor sphincters, or bad spine - this is the most frustrating part of colorectal care
The surgeon's obligation is to give the best anatomic repair possible, even though some patients will not achieve bowel control due to associated malformations
Anesthesiologist must avoid muscle relaxation before marking sphincters during PSARP, or the strength of the muscle complex will be underestimated
During PSARP opening, use Lone Star retractors and go slowly through layers rather than rushing with Weitlander retractor, which can destroy the midline and lead to misplaced anoplasty
In redo anorectal malformation evaluations, both pelvic MRI and cystoscopy are mandatory because MRI can miss roof (remnant of original fistula) if no urine present, and cystoscopy can miss it if urothelium has healed over
Remnant of original fistula (roof) can cause dribbling of urine after void, mucus in urine, stone formation, and in one case adenocarcinoma in a 30-year-old
For female perineal fistula with hole inside sphincter ellipse but anteriorly placed, posterior wall mobilization only (glorified cutback) may be adequate if hole is appropriately sized
A normal-appearing anus in a female infant shows 360-degree wrinkles around the anus, indicating muscle complex is present; if stretchable to 10 millimeters in term baby, no surgery is needed
In a 6-month-old infant, the anus should accommodate 13-14 millimeters without excessive stretching; the ARNET consortium standard is 10 millimeters for term babies
If a patient with Hirschsprung disease and ileostomy develops enterocolitis, the stoma must be blamed because there should be no obstruction through a low-pressure system unless something is mechanically or pathologically wrong with the stoma
The defunctionalized colon in Hirschsprung disease with ileostomy can develop toxic megacolon from bacterial overgrowth, even though it is distal to the stoma
Some total colonic Hirschsprung patients with ileostomy who are not ready for pull-through but have persistent distention and poor weight gain may need colectomy with Hartmann's pouch until they grow and are ready for definitive repair
For total colonic Hirschsprung disease, pull-through can be performed at 6-8 months of age if the patient is growing well and ileostomy output is thick; waiting until toilet training is not necessary
In total colonic Hirschsprung disease, families should start doing colonic irrigations once weekly or biweekly even with ileostomy in place to prevent enterocolitis and train for post-operative management
For total colonic Hirschsprung pull-through, ileoanal anastomosis is preferred; if doing ileoDuhamel, make sure the pouch is very short to avoid stasis