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Dr. Todd Ponsky

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Bowel Management for Hirschsprung's Disease Patients: Pediatric Bowel...

Video Published 2019-01-11 Updated 2026-06-10

Timestops (5)

Surgical Technique: Preserving the Anal Canal

Detailed description of transanal full-thickness resection technique for Hirschsprung disease, emphasizing preservation …

Postoperative Protocol and Enterocolitis Management

Discussion of diagnostic workup for patients with fecal incontinence after Hirschsprung surgery, including contrast enem…

Rectal Irrigation Technique

Instructional video on performing colonic irrigation for enterocolitis, demonstrating catheter insertion, saline instill…

Complications: Diaper Rash and Anal Canal Damage

Discussion of severe diaper rash in patients with fecal incontinence, particularly those with diarrhea tendency. Visual …

Total Colonic Aganglionosis Management

Protocol for total colonic aganglionosis: total colectomy with ileorectal anastomosis and protective ileostomy, with del…

Topic Overview

A surgical lecture on Hirschsprung disease management emphasizing preservation of the anal canal during pull-through operations to prevent fecal incontinence. The speaker describes a transanal full-thickness resection technique that protects the 2 cm anal canal above the pectinate line, contrasts it with endorectal dissection approaches, and details a protocol for enterocolitis prevention using rectal irrigations rather than enemas. The discussion covers complications (preventable and non-preventable), management of total colonic aganglionosis with delayed ileostomy closure until toilet training, and the importance of experienced pathology support for intraoperative frozen sections.

Key Takeaways

Preserve the 2cm anal canal above pectinate line during pull-through to maintain sensation and prevent fecal incontinence. (0:12)
Rectal irrigations, not enemas, prevent enterocolitis deaths; teach all families and start at first suspicion of symptoms. (16:49)
For total colonic aganglionosis, delay ileostomy closure until toilet-trained (>3 years) to avoid severe diaper rash. (30:56)
Resect dilated ganglionic bowel to prevent constipation; dilated segments lack normal peristalsis despite ganglion cells. (14:11)
Fecal incontinence, stenosis, and fistulas are preventable technical errors; enterocolitis is non-preventable. (13:35)

Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Speaker 1 — guest
Speaker 2 — guest
Speaker 3 — host

Chapters

0:01Surgical Technique: Preserving the Anal Canal — Detailed description of transanal full-thickness resection technique for Hirschsprung disease, emphasizing preservation of the 2 cm anal canal above the pectinate line to maintain sensation and continence. Includes video demonstration of the operative approach using Lone Star retractor and two-layer anastomosis.
11:15Postoperative Protocol and Enterocolitis Management — Discussion of diagnostic workup for patients with fecal incontinence after Hirschsprung surgery, including contrast enema and examination under anesthesia. Introduction of proactive enterocolitis prevention protocol using rectal irrigations and metronidazole, with gradual weaning based on clinical response.
19:22Rectal Irrigation Technique — Instructional video on performing colonic irrigation for enterocolitis, demonstrating catheter insertion, saline instillation technique, and drainage assessment. Emphasis on irrigation before seeking emergency care.
25:23Complications: Diaper Rash and Anal Canal Damage — Discussion of severe diaper rash in patients with fecal incontinence, particularly those with diarrhea tendency. Visual examples of destroyed anal canals from technically deficient operations.
28:00Total Colonic Aganglionosis Management — Protocol for total colonic aganglionosis: total colectomy with ileorectal anastomosis and protective ileostomy, with delayed closure until age 3+ when patient is toilet-trained for urine and accepts rectal irrigations. Critique of pouch procedures (Martin, Kimura) due to bacterial overgrowth and secretory diarrhea.

Key claims

0:12The anal canal from 2 centimeters above the pectinate line is the most sensitive part of the body, capable of distinguishing between gas, liquid, and solid — Speaker 1
0:39Damaging the anal canal results in poor sensation and fecal incontinence — Speaker 1
1:15The institution performs more reoperations for Hirschsprung disease than primary operations — Speaker 1
1:43Full-thickness rectal dissection staying close to the bowel wall prevents damage to pelvic structures including nerves and bladder — Speaker 1
1:57The endorectal dissection technique was created by Dr. Franco Suave to avoid complications that occurred when surgeons tried to reproduce Dr. Swenson's operation — Speaker 1
2:55Biopsies are taken every 5 centimeters during resection and sent for frozen section pathology — Speaker 1
3:08Not all board-certified pathologists have experience with Hirschsprung disease frozen sections; specific expertise is required — Speaker 1
5:02Traction creates the dissection plane; without traction there is no plane and no good dissection — Speaker 1
5:4280% of the time the transanal approach can reach normal ganglionic bowel; 20% require laparoscopy or laparotomy — Speaker 1
7:06The institution has performed 125 transanal resections — Speaker 1
11:28Patients with Hirschsprung disease ages 4-7 years old with fecal incontinence undergo contrast enema to determine if they have constipation with overflow pseudo-incontinence or hypermotility with non-dilated colon — Speaker 1
12:28Patients with intact anal canal and severe constipation may achieve continence with appropriate laxative management — Speaker 1
12:51Patients with intact anal canal and diarrhea tendency may achieve control with constipating diet, Imodium, 3 meals per day, and special fiber to bulk stool — Speaker 1
13:11Patients without an intact anal canal will require bowel management for life — Speaker 1
13:35Enterocolitis in Hirschsprung disease is non-preventable; the mechanism is unknown and involves bacterial overgrowth — Speaker 1
14:11Constipation after Hirschsprung surgery is partially preventable by resecting the dilated ganglionic segment, as dilated bowel lacks normal peristalsis — Speaker 1
14:58Preventable complications include dehiscence, stenosis, retraction, fistula formation (vaginal, urinary tract), and fecal incontinence from anal canal destruction—all are technical surgical errors — Speaker 1
15:53Enterocolitis can cause C. difficile infection with toxin release leading to death — Speaker 1
16:23After pull-through without colostomy, patients are kept in hospital with serial X-rays and rectal irrigations started at first suspicion of enterocolitis — Speaker 1
16:49Rectal irrigation is the most valuable life-saving maneuver in Hirschsprung disease; all mothers should learn the technique — Speaker 1
17:12Enemas can worsen enterocolitis and may cause bowel perforation; irrigation is the correct technique — Speaker 1
17:59Post-operative protocol: discharge with 3 irrigations per day and Flagyl, taper at 1 month if X-ray and growth normal, further taper at 2 months — Speaker 1
18:40Using this proactive irrigation protocol, the institution has not lost a patient to enterocolitis — Speaker 1
25:40Parents should perform irrigation at home before seeking emergency care, as emergency departments often lack supplies and care is delayed — Speaker 2
28:06Dr. Lester Martin pioneered treatment for total colonic aganglionosis at Cincinnati Children's Hospital, creating a pouch using ganglionic bowel for water absorption — Speaker 1
29:57Pouch procedures (Martin, Kimura) for total colonic aganglionosis cause bacterial proliferation and secretory diarrhea when stool is retained — Speaker 1
30:34Preferred approach for total colonic aganglionosis: total colectomy with ileorectal anastomosis above pectinate line and protective ileostomy — Speaker 1
30:56Ileostomy is maintained until patient is toilet-trained for urine, typically over 3 years old — Speaker 1
31:40Closing ileostomy in infants results in severe diaper rash because babies do not attempt to hold liquid stool — Speaker 1
32:07Ileostomy closure criteria: toilet-trained for urine, child can communicate need to use toilet, accepts rectal irrigations without distress — Speaker 1
33:01When anal canal is preserved and ileostomy closed in toilet-trained child, patient achieves stool training within 3 days — Speaker 1
32:18Patients with total colonic aganglionosis have very high incidence of enterocolitis — Speaker 1
34:03Patients without colon cannot have enemas because small bowel absorbs nutrients and cannot be cleaned or stopped between enemas like colon — Speaker 1

Open questions

Why do patients with Hirschsprung disease develop enterocolitis? What causes the bacterial overgrowth?
Why do some patients develop constipation after technically adequate resection of dilated ganglionic bowel?
What is the optimal timing for ileostomy closure in total colonic aganglionosis—should quality of life considerations outweigh earlier surgical completion?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Anal Canal Preservation and Irrigation Protocol in Hirschsprung Disease

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Teaching arc · AI-written, human-reviewed

Foundational Principle: The Anal Canal as Sensory Organ

The anal canal from 2 centimeters above the pectinate line is the only tissue in the body capable of distinguishing gas, liquid, and solid 0:12. Destroying this structure guarantees fecal incontinence regardless of how well the rest of the operation is performed 0:39. The teaching point is not that preservation matters — every surgeon knows that — but that the anal canal must be actively protected throughout the dissection, not simply avoided. The technique uses eight hooks placed at the anal-cutaneous junction, then repositioned to grasp the pectinate line itself, folding the entire anal canal out of the operative field before any rectal dissection begins. Traction sutures are then placed 2 centimeters above the pectinate line to create uniform tension during full-thickness rectal dissection 0:12 1:43.

Pathology Expertise Is Not Transferable

Board certification in pathology does not confer competence in Hirschsprung frozen sections 3:08. The institution performs biopsies every 5 centimeters during resection and sends them for frozen section analysis 2:55, but this protocol is only valid if the pathologist has specific experience with Hirschsprung histology. The error is assuming that because a hospital has a pathology department, that department can reliably interpret intraoperative Hirschsprung specimens. It cannot, unless the individual pathologist has done it repeatedly. Verify your pathologist's specific experience before relying on frozen sections to guide resection margins.

Irrigation Is Not an Enema

The terms are not interchangeable, and the distinction is life-saving 16:49 17:12. Irrigation involves instilling saline and allowing it to drain back out immediately, clearing the colon without retention. Enemas retain fluid in diseased bowel and can cause perforation 17:12. The discussant emphasizes that many academic institutions use the terms indiscriminately, which is wrong. Parents should be taught to irrigate at home before seeking emergency care, because emergency departments often lack supplies and care is delayed 25:40. The protocol after primary pull-through without colostomy: three irrigations per day plus metronidazole, taper at one month if X-ray and growth are normal, further taper at two months 17:59. Using this proactive approach, the institution has not lost a patient to enterocolitis 18:40.

Constipation Phenotypes Determine Management

A 4- to 7-year-old with fecal incontinence after Hirschsprung surgery requires contrast enema to distinguish constipation with overflow pseudo-incontinence from hypermotility with non-dilated colon 11:28. If the anal canal is intact and the patient is severely constipated, appropriate laxative management may achieve continence 12:28. If the anal canal is intact but the patient has diarrhea tendency, constipating diet, loperamide, three meals per day, and bulking fiber may achieve control 12:51. If the anal canal is destroyed, none of this matters — the patient will require bowel management for life 13:11. The teaching point: do not waste time with medical management in a patient with no anal canal. The contrast enema tells you whether you have something to work with.

Total Colonic Aganglionosis: Timing Over Technique

The preferred approach is total colectomy with ileorectal anastomosis above the pectinate line and protective ileostomy 30:34. The ileostomy is maintained until the patient is toilet-trained for urine, typically over 3 years old 30:56. Closing the ileostomy in infancy results in severe diaper rash because infants do not attempt to hold liquid stool 31:40. The closure criteria are specific: toilet-trained for urine, able to communicate the need to use the toilet, and accepts rectal irrigations without distress 32:07. When these criteria are met and the anal canal is preserved, the patient achieves stool training within three days 33:01. The discussant acknowledges that parents and surgeons dislike waiting three years, but emphasizes that the protocol serves the patient's quality of life, not the convenience of doctors or parents. Patients without a colon cannot have enemas because small bowel absorbs nutrients and cannot be cleaned or stopped between enemas like colon 34:03.

Takeaways from this story

Anal canal preservation requires active protection: fold it out of the field with repositioned hooks before starting rectal dissection.
Board-certified pathologists cannot reliably interpret Hirschsprung frozen sections without specific experience in this disease.
Irrigation (instill and drain) prevents enterocolitis; enemas (retain fluid) can perforate diseased bowel—the terms are not interchangeable.
Contrast enema distinguishes overflow pseudo-incontinence from true hypermotility; intact anal canal with constipation may respond to laxatives.
Total colonic aganglionosis: maintain ileostomy until toilet-trained for urine (>3 years); stool training occurs within 3 days after closure.

Anal Canal Preservation and Irrigation Protocol in Hirschsprung Disease

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Teaching arc · AI-written, human-reviewed

Teaching Hirschsprung Surgery as Tissue Preservation

The discussion opens with a principle that reframes the entire operation: the anal canal from 2 centimeters above the pectinate line is the most sensitive tissue in the body, capable of distinguishing gas from liquid from solid 0:12. Damage this structure and the patient has permanent fecal incontinence regardless of how well you resect the aganglionic segment 0:39. The institution now performs more reoperations for Hirschsprung disease than primary cases, most of them correcting preventable technical errors 1:15.

The transanal full-thickness approach protects what matters. The endorectal dissection was invented to avoid the complications surgeons encountered trying to reproduce Swenson's original operation 1:57, but staying in the submucosal plane does not inherently protect pelvic structures. Full-thickness rectal dissection close to the bowel wall avoids injury to nerves and bladder 1:43. The technique begins with eight hooks at the anal-cutaneous junction, then repositioned to grasp the pectinate line, folding and protecting the anal canal by definition. Traction sutures placed 2 cm above the pectinate line create uniform tension — one of the discussants emphasized that traction creates the plane, and without traction there is no plane and no good dissection 5:02. Biopsies are taken every 5 centimeters and sent for frozen section 2:55, but only if your pathologist has specific experience with Hirschsprung histology; board certification alone is insufficient 3:08. Eighty percent of cases can be completed transanally; twenty percent require laparoscopy or laparotomy 5:42.

Irrigation is not an enema, and the distinction saves lives. Rectal irrigation — instilling saline and allowing it to drain back out — is the most valuable life-saving maneuver in Hirschsprung disease, and all mothers should learn the technique 16:49. Enemas retain fluid and can worsen enterocolitis or cause perforation in sick bowel 17:12. Many academic institutions use the terms interchangeably, which is wrong. After pull-through without colostomy, patients are kept in hospital with serial X-rays and irrigations started at the first suspicion of enterocolitis 16:23. The discharge protocol is aggressive: three irrigations per day plus metronidazole, tapered at one month if X-ray and growth are normal, further tapered at two months 17:59. Using this proactive approach, the institution has not lost a patient to enterocolitis 18:40. Parents should irrigate at home before seeking emergency care, as emergency departments often lack supplies and care is delayed 25:40.

Constipation versus hypermotility determines the management path. For patients ages 4-7 with fecal incontinence after Hirschsprung surgery, contrast enema determines whether they have constipation with overflow pseudo-incontinence or hypermotility with a non-dilated colon 11:28. If the anal canal is intact and the patient is severely constipated, appropriate laxative management may achieve continence 12:28. If the anal canal is intact but the patient has diarrhea tendency, a constipating diet, loperamide, three meals per day, and bulking fiber may achieve control 12:51. If the anal canal is destroyed, the patient will require bowel management for life regardless of colonic function 13:11.

Total colonic aganglionosis requires patience, not ingenuity. Pouch procedures using ganglionic bowel for water absorption cause bacterial proliferation and secretory diarrhea when stool is retained 29:57. The preferred approach is total colectomy with ileorectal anastomosis above the pectinate line and protective ileostomy 30:34, maintained until the patient is toilet-trained for urine — typically over three years old 30:56. Closing the ileostomy in infants results in severe diaper rash because babies do not attempt to hold liquid stool 31:40. Closure criteria are specific: toilet-trained for urine, able to communicate the need to use the toilet, and accepts rectal irrigations without distress 32:07. When the anal canal is preserved and the ileostomy closed in a toilet-trained child, stool training occurs within three days 33:01. Patients without a colon cannot have enemas because small bowel absorbs nutrients and cannot be cleaned or stopped between enemas like colon 34:03.

Preventable complications are technical errors. Enterocolitis is non-preventable; the mechanism is unknown and involves bacterial overgrowth 13:35. Constipation is partially preventable by resecting the dilated ganglionic segment, as dilated bowel lacks normal peristalsis 14:11. But dehiscence, stenosis, retraction, fistula formation to vagina or urinary tract, and fecal incontinence from anal canal destruction are all preventable — they are technical surgical errors 14:58. The discussant emphasized: "We are trying to benefit the patient, not the doctors and not the parents."

Takeaways from this story

Anal canal preservation from 2 cm above pectinate line is non-negotiable—damage this tissue and continence is lost permanently.
Irrigation (instill and drain) prevents enterocolitis; enemas (retain fluid) can perforate sick bowel—the terms are not interchangeable.
Proactive irrigation protocol (3x daily post-op, tapered over 2 months) has eliminated enterocolitis mortality at this institution.
Total colonic aganglionosis: maintain protective ileostomy until toilet-trained for urine (>3 years), then close—stool training follows in 3 days.
Dehiscence, stenosis, retraction, and fistula formation are preventable technical errors, not inherent disease complications.

Preserving Continence After Hirschsprung Surgery: Why Technique Determines Outcome

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Explainer · AI-written, human-reviewed

Why This Subspecialty Exists

Hirschsprung disease requires removing aganglionic bowel while preserving the mechanisms of fecal continence—a technical challenge that sits at the intersection of neonatal surgery, colorectal reconstruction, and long-term functional outcomes. The field exists because early surgical approaches, while addressing the underlying pathology, often destroyed the anal canal and left children with lifelong incontinence. This discussion addresses the technical and postoperative decisions that determine whether a child achieves normal bowel control.

The Core Problem

The anal canal—specifically the 2 cm segment above the pectinate line—is the most sensitive tissue in the body, capable of distinguishing gas from liquid from solid 0:12. Damage this structure during pull-through surgery and the patient loses both sensation and sphincter function, resulting in permanent fecal incontinence 0:39. The institution presenting this protocol performs more reoperations for Hirschsprung disease than primary operations, a ratio that reflects the frequency of technical failures elsewhere 1:15.

The challenge is geometric: you must resect aganglionic rectum, reach normal ganglionic bowel (which may be proximal), and anastomose to the anal verge—all without injuring the 2 cm canal that controls continence. Eighty percent of cases can be completed transanally; twenty percent require laparoscopy or laparotomy when the transition zone is too proximal 5:42.

How the Approach Works

Operative technique. The transanal full-thickness resection begins with eight hooks placed at the anal-cutaneous junction, then repositioned to grasp the pectinate line, folding and protecting the anal canal. Traction sutures are placed 2 cm above the pectinate line to provide uniform tension during dissection 5:02. The rectum is divided full-thickness, staying close to the bowel wall to avoid pelvic nerves and bladder 1:43. Biopsies are taken every 5 cm and sent for frozen section to confirm the resection reaches ganglionic bowel 2:55.

This differs from the endorectal dissection technique, which was developed specifically to avoid complications surgeons encountered when attempting to reproduce Swenson's original operation 1:57. The full-thickness approach relies on the principle that staying immediately adjacent to bowel wall protects all surrounding structures—a lesson borrowed from anorectal malformation surgery.

Pathology requirements. Board certification alone does not qualify a pathologist to read Hirschsprung frozen sections 3:08. Surgeons must verify their pathologist has specific experience with this disease before relying on intraoperative guidance. This is not a minor point—resecting too little bowel leaves aganglionic tissue; resecting too much risks a tethered, poorly mobile pull-through.

Postoperative protocol. After pull-through without colostomy, patients remain hospitalized with serial abdominal X-rays. At the first sign of enterocolitis—distention, poor feeding, increased bowel sounds—rectal irrigations are started 16:23. Patients are discharged on three irrigations daily plus metronidazole, tapered at one month if X-ray and growth are normal, further reduced at two months 17:59. Using this proactive protocol, the institution has not lost a patient to enterocolitis 18:40.

Irrigation vs. enema. This distinction matters clinically. Irrigation involves instilling saline and allowing it to drain back out immediately, clearing bacterial overgrowth without retention. Enemas retain fluid and can perforate sick bowel 17:12. Parents are taught to irrigate at home before seeking emergency care, as emergency departments often lack supplies and delay treatment 25:40.

Assessing Salvageability

When a child presents years after pull-through with fecal incontinence, contrast enema determines whether the colon is dilated (constipation with overflow pseudo-incontinence) or non-dilated with hypermotility (true incontinence) 11:28. If the anal canal is intact and the patient is constipated, appropriate laxative management may restore continence 12:28. If the canal is intact but the patient has diarrhea tendency, constipating diet, loperamide, three structured meals daily, and bulking fiber may achieve control 12:51. If the anal canal was destroyed during the original operation, the patient will require bowel management for life 13:11.

Total Colonic Aganglionosis

This variant requires total colectomy with ileorectal anastomosis above the pectinate line and protective ileostomy 30:34. The ileostomy is maintained until the child is toilet-trained for urine—typically over three years old—and accepts rectal irrigations without distress 30:56. Closing the ileostomy in infancy produces severe diaper rash because infants make no effort to retain liquid stool 31:40. When the anal canal is preserved and the ileostomy closed in a toilet-trained child, stool training occurs within three days 33:01.

Pouch procedures (Martin, Kimura) using ganglionic bowel for water absorption cause bacterial proliferation and secretory diarrhea when stool is retained 29:57. Patients without a colon cannot have enemas because small bowel absorbs nutrients continuously and cannot be cleaned or stopped between enemas like colon 34:03.

When to Involve This Team

Refer when a child with prior Hirschsprung surgery has persistent fecal incontinence, recurrent enterocolitis despite standard management, or when you are uncertain whether the anal canal was preserved. Refer before primary pull-through if your pathologist lacks specific Hirschsprung frozen section experience. For total colonic aganglionosis, early referral allows coordinated planning of the staged reconstruction and family education about the prolonged ileostomy.

What Remains Contested

Enterocolitis mechanism is unknown; bacterial overgrowth occurs but the trigger remains unclear 13:35. Constipation after anatomically successful pull-through occurs unpredictably even when dilated ganglionic bowel is resected 14:11. The optimal timing for ileostomy closure in total colonic aganglionosis is debated—this protocol prioritizes toilet training over early closure, accepting years of ostomy management to avoid severe diaper rash and achieve rapid stool training.

Takeaways from this story

The 2 cm anal canal above the pectinate line distinguishes gas, liquid, and solid—damage it and the patient has lifelong incontinence.
Rectal irrigation (instill and drain immediately) prevents enterocolitis; enemas (retained fluid) can perforate sick bowel.
Board-certified pathologists without Hirschsprung frozen section experience cannot reliably guide intraoperative resection margins.
In total colonic aganglionosis, delay ileostomy closure until toilet-trained for urine (age 3+) to avoid severe diaper rash and achieve rapid stool training.

Keywords

Hirschsprung disease fecal incontinence enterocolitis total colonic aganglionosis neurogenic bladder diaper rash C. difficile infection constipation overflow pseudo-incontinence transanal full-thickness resection endorectal dissection Swenson operation pull-through laparoscopy laparotomy

Hashtags

#HirschsprungDisease #PediatricSurgery #FecalIncontinence #SurgicalTechnique #EndorectalDissection #TransanalResection #BowelManagement #AnalCanal #SphincterMechanism #Enterocolitis #Constipation #SurgicalComplications #Pathology #NeurogenicBladder #Biopsy #Peristalsis #RectalWall #SurgicalTraining #FrozenSection #PelvicStructures #ColorectalSurgery

Transcript

But in addition, if we damage the mechanisms of bowel control, the patient is going to be fecally incontinent. All the operations designed to treat Hidbune disease. Emphasize the need, the, the importance of preserving the anal canal, preserving the anal canal meaning from 2 centimeters above depicting it lying down because the anal canal represents the most sensitive part of our body. We can recognize in that area the difference between gas, liquid, and solid. There is no other tissue in our body that can have that, that can do that. So if we damage the anal canal, the patient will have no sensation or poor sensation, and that means fecal incontinence. In addition, damaging the anal canal most likely represents the possibility that the sphincter was also damaged because the sphincter is located surrounding the anal canal. So that's why it's so important for us professors of pediatric surgery to be particularly obsessive about teaching our fellows and residents to preserve the anal canal in all these operations. We happen to have more reoperation for his spoon than primary his prunes to consider all the errors and problems that are provoking hispne disease and I have the next slide. Our sur surgical approach for Hib disease consists in doing a trans anal full thickness resection. In other words, you know that it's very popular to do a submucosal endorectal dissection. We prefer to do full thickness resection because we have learned in anorectal malformations that that if you stay very close to the bowel wall, you don't damage any structures in the pelvis. The end of rectal dissection was was uh designed for Hishpun disease but by Doctor Franco Suave because when Doctor Swenson published on his paper about the surgical treatment for Hishmun disease when people try to reproduce the operation of Doctor Swenson. Many problems occur. Many patients suffer from damage of important pelvic structures, nerves, neurogenic bladder, vagina, other neighbor structures. And in order to avoid that, not everybody could be so accurate as Doctor Swenson. Then Doctor Franco Suave created this endorectal dissection. But I believe that if you dissect the rectum close enough to the rectum, you respect everything and the patients don't have neurogenic bladder or any of those problems and we have done that many times. We do it all the time in the rectum. That's why we do a trans anal resection but full thickness rectum. And we go taking biopsies every 5 centimeters sending to pathology department but of course that you can do that provided you have an excellent pathology department. A big mistake and wrong generalization in pediatric surgery all over the world, including United States, is to believe that because there is a department of pathology, they, they, and because you have board certified pathologists they will be able to tell you the right answer in Hishbu disease that is grown. You need a pathologist that has experience with Hishman's disease and then. There are pathologists that have experience for frozen sections. There are pathologists that have no experience for frozen section. There are pathologists that have experiences with the hematoxyline neocene. So you have to, to, to find out what kind of experience your pathologist has. So this technique in which we are taking biopsies every 5 centimeters, send it to pathology for frozen section. Can only be implemented if your pathologist has experienced with frozen sections. Otherwise, otherwise you have to implement your own methodology treatment here you are seeing an image of the beginning of an operation for his bone disease. We use eight hooks that that separate the, the edges of the anus, and you can see they're depecting it line. So the distance between the skin and depicting a line, that's what is what we call anal canal that's is extremely important to preserve and to be sure that we preserve what we do after you see after you we put these hooks like you see there, we move those hooks deep into the to grab the pectinate line. At the next you see now those hooks are an independenting line. So now you are, you are seeing only rectal mucosa. All the anal canal is now folded and protected by definition. And then from there we go 2 centimeters deeper to put a series of stitches to in order to apply uniform traction to facilitate the dissection of the rectum. Remember, traction creates the plane. No traction, no plane, no plane, no good dissection. No good dissection. No good surgery. No good surgery. Complications. Complications. Bad reputation. Bad reputation. No patience. No patience, no money, no money, no wife. So you better put distinction traction. So you apply the traction and then start this is the dividing the rectum full thickness there. They want you to use the pointer and oh, I can, yeah, I can help with that next. Mark, can you give me the pointer? Yeah OK, oh, it's a video. It's a video, so you will see a very short video of the way we do this, uh, we the, the beginning of the operation is the crucial part, and, and 80% of the times through the transcendent approach you can reach the normal ganglionic bowel. Um, 20% of the time we have to go through the abdomen with laparoscopy or laparotomy depending on your preferences and your skills. They're loading up the video. So, in the meantime, I see that there is a question. You tell, oh, the video is ready? Yeah, it's playing. Go ahead. This slides show the normal rectum, normal anal canal and sphincter mechanism. The trans anal resection of the rectosigmoid must be performed dividing the rectum 2 centimeters above depicting at 9. The external appearance of these patients perineum shows a patulous anus and an extremely severe rash consecutive to fecal incontinence. This slide shows the anus of a patient that had a technically deficient transanal resection of the rectosigmoid. The resection unfortunately included the entire anal canal and the pull through bowel was anastomos to the perianal skin. The patient suffered from permanent fecal incontinence. We have done 125 trans anal resections. This video will show what we consider the essential steps of the operation to preserve the continent's mechanism. We strongly recommend the use of a lone star retractor. The 8 hooks are placed circumferentially at the end of cutaneous junction. Depicting line is exposed. The next step consists in replacing the hooks of the retractor, taking rectal mucosa in order to hide and protect the pectinate line. A series of fine sutures are placed in a circumferential manner higher than 2 centimeters above the pectinate line. Applying uniform traction on the sutures, a circumferential incision of the rectal wall is started. The dissection of the rectum is performed, remaining in intimate contact with the rectal wall, dividing and burning all the extrinsic blood supply of the rectum. Remaining in intimate contact with the rectal wall prevents damage to important nerves and pelvic structures. In order to do this, all the fat tissue must be dissected away from the rectum. Once we have reached the desired level of resection, a two layer anastomosis is created between the pull through bowel and the rectal wall. Here you can see the first couple of stitches of the outside layer. The sutures are placed by quadrants followed by stitches in between. This is the anterior stitch. And the lateral stitch. The outside layer has been finished. The rectal wall is divided and the inner layer suture line is started again by quadrants. This is a lateral stitch. This is the posterior stage. The anterior one. And the last lateral stitch. Then the sutures in between the quadrants are placed. The hooks after these are removed following this principle, the sensation area of the anal canal and the sphincter mechanism are preserved, which guarantees bowel control. Yeah. Well, I hope you, uh, were able to see the important details to to preserve the anal canal and for as part of our. As part of our protocol of study of patients that come to our clinic, patients who were born with Houston disease were operated for Houston disease, the patients are 457 years old and fecally incontinent. We have a specific protocol for them. We order a contrast enema to see if the patient belongs to a constipated patient with fecal incontinence or hypermotility non-dilated colon with fecal incontinence. And in addition, we put the patient to sleep to see if the patient has an intact anal canal or the anal canal has been destroyed. And we have an enormous number of patients with a completely damaged anal canal. When we see that there is no anal canal, when the surgeons pull the bowel and suture it basically to the skin, you can say that the patient is going to have fecal incontinence for life. It's going to be, is going to receive bowel management for life. If you see the anal canal intact and the patient is very constipated, then you have a reason to believe that perhaps actually the patient is suffering from overflow pseudo incontinence and with laxative with the right amount of laxative, the patient may behave like a continent patient. If the patient has tendency to diarrhea, have constant very liquid stools. And intact anal canal by giving constipating diet and Imodium, chances are that the patient will be able to control. And in addition, we give them 3 meals per day and a special fiber to make the stool bulky. But if the patient has no anal canal, don't waste your time with laxatives and all that, because the patient will be incontinent, most likely needs bowel management and the bowel management, the type of bowel management will be discussed, uh, later today. Um Next night. Next month? Yeah. So the complications and the complications and sequela in patients with his spoon disease, I personally like to divide it into preventable, non-preventable, which is enterocolitis. We don't know why patients have enterocolitis. It's a kind of a mystery we are looking forward to. The new generation of pediatric surgeons with the knowledge and dedication to research, basic science research to tell us what's wrong with these patients that developed this, they, they grow bad bacteria in the colon and they have this terrible complication that may kill patients. Um, then there is something there or constipation, I believe that is partially, partially, uh, preventable because we have learned that in fishbone disease we are obligated to resect the, the ganglionic segment of bowel but also to. Resect the normal ganglionic dilated piece of bowel because if you do a pull through with a very dilated piece of bowel that piece of bowel dilated doesn't have a normal peristalsis and the patient will suffer from constipation. But even following these rules, some patients have constipation. I don't know why, and that's why I call this partially preventable and then becomes the preventable preventable complications, complications that should never occur. The complications that are our responsibility, which is dehesence stenosis. Retraction, fistula formation, fistulas to the vagina to the urinary tract, all those are technical errors from the surgeon, so that should never happen. We should, uh, we should train our fellows to avoid that kind of problems. And finally, fecal incontinence because of destruction of the anal canal. If you look into the literature about. About the publications for his disease you will not find many publications discussing this subject because we don't like to talk about that but it's extremely important. So, as you know, enterocolitis, I don't have to say much about that, but you have seen these patients with Hirschp disease. They suffer after an operation, after a technically good operation, the patients suffer from abdominal distention, bacterial proliferation, sometimes, specifically, they develop, um, C. difficile and release of toxins and death, so. We are very obsessive about the prevention and treatment of the uh of this complication. We are very proactive in the management of this. So we, when we do a pull through in a patient with Hodgkin disease, most of our patients, we do it without a colostomy, but we keep the patient. In the hospital taking X-ray films, looking at the radiologic image of the bowel, and we don't hesitate to start irrigations of the rectum to as soon as we suspect that the patient is developing enterocolitis. We don't want to wait until the baby is extremely sick. And we teach the mother how to do rectal irrigation because rectal irrigation is the most valuable maneuver saving lives maneuver in patients with Hisb disease and all mothers should learn how to do rectal irrigation. You will be surprised how many institutions, very academic, famous institutions, confuse the, the, the, and they don't know the. Difference between enemas and irrigations and they use both terms in an indistinct way and that's wrong because if you give enemas to a patient with enterocolitis you may make making the patient worse in fact you may produce a perforation of the bowel the bowel is very sick so irrigation we you will see a video that illustrates what irrigation means for us. And our nurses are experts in doing that and we teach the mothers and then the mothers learn when the baby needs an irrigation very early. They don't wait until the baby is very sick. The child is not eating well, looks a little distended, makes a lot of noise in the bowel. The mother does the irrigation and the patient feels much better. So we are very, and we also give metronidazole in addition to the irrigation. So specifically when a patient has a huge operation in our institution. We discharge the patient with indications, proactive indications, prophylactic, 3 per day and and Flagyl, and a month later, the patient comes back to the clinic. We take an X-ray film. If the gas pattern in the abdomen looks normal, if the child is eating and growing normally, we decrease the number of irrigations to 2 per day and decrease the amount of Flagyl 50%. And then a month later it comes back, another X-ray film. The baby's doing well. Again, we decrease the number of irrigations to one per day and decrease the amount of Flagyl. And by doing that, we have, we have not lost one patient from enterocolitis. Want to see the video. Yeah, before we play the video, let me just make one thing clear to everyone. Um, I know that. With our new technology now, the faculty is a little confused. All the faculty out there, um, we have not brought your cameras in until we're ready for the panel discussion. So in about 5 minutes, we're gonna be bringing all of you in, uh, to talk and share what's going on and answer questions. So we're bringing all your cameras in. We can see you, but the rest of the world cannot see you yet. So, uh, as soon as we're ready for the panel discussion, right now, we're gonna roll the video. If your child has been diagnosed with entercolitis, we recommend that irrigation should be done 3 times a day and more often if needed. If your child has entercolitis symptoms of fever, belly distention, not stooling, vomiting, explosive diarrhea, foul smelling stool or gas, you should irrigate first, then seek medical attention immediately. In order to perform a colonic irrigation, you will need to gather some supplies. You will need normal saline. A few bath towels or washcloths. Two basins or small tubs. A 60 mL syringe with a catheter tip. Lubrication that is water-soluble. A silicone catheter you will need a 16 French catheter if your child is under 1 year of age or a 24 French catheter if your child is over 1 year of age. Start by warming the normal saline. It is suggested to sit the bottle of saline in a sink of warm water. It is very important to test the temperature of the saline on your wrist to ensure that it is not too hot. Pour the warm saline into one of your basins. Lubricate the appropriate size catheter and gently insert it into the rectum. Pause and allow for any stool or gas to run out the end of the catheter into the basin. As you advance the catheter, allow for any other pockets of stool or gas to empty. If gently pushed, the catheter should follow the curve of the colon. It is important not to force or advance the catheter further than the wide divider port of the catheter. Note that the best position is to have your child laying on their back with their knees bent to their chest. Draw up 20 mLs of warm saline into your catheter tip syringe. Connect the syringe into the end of the catheter and inject the saline into the catheter. Disconnect the syringe from the end of the catheter and allow for the saline solution to drip into an empty basin. You will repeat this process by advancing the catheter about 1 inch each time before injecting 20 mLs of warm saline. It is important that between each installation of saline to allow for the solution to drain from the catheter into the basin. If the amount that is draining out is not equal to or more than the amount of saline that you put in, continue to move the catheter in and out while twisting to drain the pockets of saline, gas, and stool. If you feel this has not allowed the colon to drain well, you can attach the syringe to the catheter and pull back very gently on the syringe. It is important not to pull on the syringe if you feel resistance. Repeat this process until the fluid that is draining back out from the catheter is clear. Once the return fluid is clear, remove the catheter from the rectum. Wash your supplies with soap and water and allow to dry. These supplies can be reused. OK, so I just wanna draw attention to something that we feel is very important that we teach our families for the irrigation um when our parents suspect um signs of entercolitis or there's distention. And they are concerned that their child needs to seek care, we always instruct the parent to give an irrigation first in the home and then go to seek attention. We often have patients who show up in an emergency room or a care office and there is not supplies there for them to perform the irrigation. And then the care is delayed and the child could be sitting for, for a long time to hours without irrigations getting very sick. So we always instruct the parents to give the irrigations before seeking care and to even take their supplies with them. Hold on. Sorry guys. OK, now it's it. Next. Go ahead. Next is. The next slide, OK. Yeah. Now, the um the um patients with fecal incontinence for Hibs disease suffer very much about severe diaper rash, particularly if they belong to the group of patients with tendency to diarrhea. And the, the, we surgeons usually don't discuss the problem of diaper rash. We let that, let that problem for the mothers and the nurses. But it's is, is, uh, the severe diaper rash like the one that you are seeing in your screen. Produces a very bad quality of life, makes the babies really suffer a lot as well as the mothers. And next. Now, this particular patient that you are seeing here has a completely damaged anal canal. Mark, yeah, um, someone keeps restarting the OK. It's a completely damaged anal canal and severe diaper rash that it's a diaper chronic diaper rash that develops areas of granulation equivalent to a second degree burn next. Here you see a patient that had an operation for his spoon and you don't see a pectinate line that means that the suture, the, the surgeon sutured the colonic mucosa to the skin basically next. Next. And these are the, the steps of a next. Now we're I'm gonna say a few words about another condition called total colonic ganglionosis. In this picture you can see um Doctor Lester Martin who made a contribution in the treatment for total colonic ganglionosis. It turns out that Doctor Lester Martin worked in at Cincinnati Children's Hospital. He trained at Boston Children's Hospital and become the pioneer of pediatric surgery in Cincinnati. So he's still um he's retired but he visits us once in a while so we had the opportunity to take this picture at our colorectal center and he is a very nice person. We have a great deal of respect for him. The idea of Doctor Martin uh talking about total coronic ganglinosis, the problem, as you know, in total coronica ganglionnosis is that we resect the entire colon and therefore these patients will have diarrhea for life. Then, um, in order to decrease the number of bowel movements in order to absorb water, in order to try to form solid stools, he came up with the idea of preserving part of the ganglionic bowel and take normal ganglionic bowel down and make a lateral lateral anastomosis between the the ganglionic and the normal ganglionic. Using taking advantage of the peristalsis of the normal ganglionic bowel and taking advantage of the water absorption capacity of the ganglionic segment, he will create a, a, a um a pouch or, or reservoir that would be able to form more solid stool. The idea was very good. Then Doctor Kimura used the same principle but using the right color. And so it's very ingenious to do that, but Mother Nature doesn't follow our plans. So every time we try to, to cheat on Mother Nature we learn that things are more complex than what we thought. So what happens is that when, when you take, when you retain stool in patients with his disease, when you have, um, holding the stool, it produces bacteria proliferation, inflammatory changes that produce secretory diarrhea. So we don't like the operation originally done by Doctor Lester Martin and Doctor Kimura or any other kind of patches of uh of patches because we have been in charge of resecting that and those patients don't do well. Uh, total coronica ganglionosis is a very serious condition, and we prefer to do an ileo direct ileoprocto anastomosis preserved in the anal canal with emphasis in the anal canal, but we have a, a, a very peculiar way to manage these patients that not everybody likes, but we are convinced that it is the best way to manage these patients. Regardless of the age of the patient, when the patient comes to us, we do a total colectomy, ileoprocto anastomosis, ileorectal anastomosis above depicinate line, and a protective ileostomy, and we leave the patient with the protective ileostomy until the patient is toilet trained for urine, which means usually more than 3 years old. So we are living in the era of competitions. Pediatric surgeons compete. If you operate on a patient, uh, 1 year old, I tried to do it 6 months. Another guy tried to do it 3 months, another 2 months, another newborn, another in utero, but we sometimes we don't take very much into consideration the quality of life of the patient. So we believe that if you close the ileostomy, uh, as you do this procedure in a, in a baby, the baby will have a terrible diaper rash even if you preserve the anal canal because the baby doesn't make any effort to hold the stool, will be passing liquid stool constantly, and the diaper rash that these patients, uh, have are the worst type that you have seen, whereas what we do is to wait until the patient is 3 years of age and comes to the clinic. And we and demonstrates us that he's toilet trained for urine. The child talks. He tells the mother, I want to use the toilet. He's accustomed to be clean in the underwear. And another condition is that she must accept rectal irrigations because patients with total coronic ganglionnosis have a very high incidence of enterocolitis. So if the patient doesn't tolerate rectal irrigations every time the mother is trying to do that in a child. That is 3 years old is gonna be a big drama in the family. So the, the, we instruct the mother to, to practice the, uh, simulate irrigations at home with a very soft catheter so the child understand that doesn't hurt. And so when the, the mother should come to the clinic with a toilet trained child for urine. And tolerating erectile irrigation, then we close the ileostomy and guess what? If you preserve the anal canal, within 3 days, the patient is totally trained for a stool. Parents sometimes don't like the idea to give a baby with an ileostomy for 3 years, and doctors don't like that idea, but actually the patient is very happy and we are trying to benefit the patient, not the doctors and not the parents. Next slide. Got it. So look at the kind this this patient total chronic ganglionosis, they did a pull through newborn baby and in addition they destroy destroyed the anal canal and the patient came like that a year or two later and is really suffering a lot next. So in our protocol, as I said, total colectomy, straight ileoprocton anastomosis, protective ileostomy next. And then a ileostomy closure only when the patient is totally trained for urine and accepts rectal irrigations. The patients without colon cannot have enemas, a small bowel um. Uh, the, um. The small bowel absorbs nutrients and there is no way to clean the small bowel and stop it from moving in between enemas like we do with the colon. Next. Um-hum. Hm. It looks like that's the last slide. That's it. So,

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