Surgical Procedures for Hirschsprung Disease
With Dr. Alberto Peña & Dr. Luis de la Torre & Dr. Richard Krauss · hosted by Dr. Todd Ponsky & Dr. Andrea Bischoff · StayCurrentMD
Cued at 126:31 · stops at 127:16 · press play
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
Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.
If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.
When starting laparoscopically, you can look at the bowel and often tell what looks normal vs. abnormal, then take a biopsy at that level.
If you start transanally and break through the peritoneum, it can be difficult to maintain pneumoperitoneum when you then go laparoscopically.
Laparoscopic dissection is easy and gives you a head start when doing the transanal portion.
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).
A bad surgeon will damage the patient both ways—laparoscopically or transanally.
The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.
Patients complain about fecal incontinence, not the size of the scar.
When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.
Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.
The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.
Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.
Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.
A two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.
Keys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.
Prone position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.
Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.
Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.
The majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.
A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.
When you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.
The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.
When we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.
Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.
After removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.
Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.
Even in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.
Fecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.
Most patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel.
Patients are born with bowel control; we provoke fecal incontinence through surgical technique.
Until the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation.
Transanal pull-through for idiopathic constipation is not a good operation.
When dissecting the anterior rectal wall transanally, the dissection must be conducted very meticulously because the rectum has a common wall with the vagina and prostatic urethra.
Cases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications.
During full-thickness transanal dissection, if you see fat around the rectum, you can get closer to the rectum because that means you are not in the real rectal wall.
The outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer.
Avoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much.
Laparoscopy for Hirschsprung is the same as laparotomy—it's the same Soave, Duhamel, or Swenson technique, just using different instruments.
In many countries where laparoscopy is not common, surgeons continue using laparotomy and patients do well—it doesn't matter if they have laparotomy or laparoscopy.
If you can do the Swenson procedure in 3 stages and the patient outcome is good, that's perfect—you don't need to do a transanal endorectal pull-through.
To do a good endorectal pull-through, you need to identify a very good plane of dissection and observe the circular fibers of the rectum that will become the rectal cuff.
If you are in the right plane of dissection during endorectal pull-through, the operation is almost bloodless.
When you leave a muscular cuff with a huge, floppy, dilated colon, the patient will most probably have chronic obstruction, which produces chronic colitis.
To create the muscular cuff, place two sutures: one taking mucosa and muscular cuff, the other taking just muscular cuff, then cut in between.
After opening the muscular cuff, you can see the mesentery from the posterior wall; ligate and cut the vascular vessels to gain more length of colon.
While waiting for frozen-section biopsy results, resect as much muscular cuff as possible and perform a myectomy on the posterior wall, resecting 1-2 cm in length and creating a short muscular cuff from below.
For frozen-section biopsies during Hirschsprung surgery, always send full-thickness biopsies, never small seromuscular biopsies, because pathologists suffer with very small biopsies on frozen section.
One of the most common problems in endorectal pull-through is leaving a large muscular cuff, which causes obstruction.
The anastomosis should be performed with the most perfect technique possible, using fine sutures (5-0 or 6-0 Vicryl).
To perform the anastomosis, remove the Lone Star retractor hooks so you can see the anal canal again and ensure proper placement.
When doing rectal irrigation for Hirschsprung, if the patient improves immediately, they are a candidate for transanal approach.
Patients with long-segment or total colonic aganglionosis do not improve with rectal irrigation.
Patients with late presentation and chronic dilation of the colon (massive megacolon) are not suitable for primary transanal pull-through.
Chronic dilation of the colon has poor motility, so these segments need to be resected.
To achieve the most accurate, functional, and anatomical surgery for Hirschsprung, you need good bowel (good irrigation, good pathology, no tension), caution in the blind zone (pelvis), and a perfect anastomosis (not too low, not too high, technically well-performed, preserving anal canal).
The anal canal is composed of three zones: anoderm (squamous epithelium), the area where the pectinate line lives, and the columnar zone.
The columnar zone should be preserved for fecal control.
Patients who have had different Hirschsprung techniques (transanal, laparoscopic, Soave, Swenson) and have none of these anal canal elements are fecally incontinent.