The Colorectal Quiz Episode 22: Hirschsprung Disease - the Soiling Patient Part 1

Published: 2021-11-01

8 Views

0 Likes

0 Shares

0 Comments

Colorectal Channel

View profile →

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

Amanda Jensen — host
Jason Fisher — guest
Mark Levitt — guest
Hira Ahmad — guest

Chapters

0:00Introduction and Framing: Soiling vs. Obstruction — The hosts introduce episode 22 on Hirschsprung's disease focusing on the soiling patient, distinguishing this from the obstructed patient discussed in the previous episode. They frame the two problematic post-pull-through presentations: obstructed patients with distention and enterocolitis versus soiling patients who are never distended but poop constantly.
3:24Setting Expectations: What to Tell Families — Discussion of what surgeons should tell families at diagnosis. Levitt explains that if the pull-through is done correctly with no anatomic problems and preserved sphincters, most patients do extremely well with only minor medical management for constipation. He emphasizes there is no reason any Hirschsprung's patient should be obstructed or soiling if properly managed.
4:57The Critical Question: Potential for Bowel Control — Levitt introduces the most important question for any soiling patient: what is the patient's potential for bowel control? He explains that Hirschsprung's patients are born with normal sphincters and intact dentate lines, giving them full potential for voluntary bowel movements—unless the dentate line is lost from dissection started too low or sphincters are overstretched.
8:10Visual Assessment of Sphincters: Normal vs. Patulous — The team reviews clinical photographs showing normal closed sphincters versus patulous sphincters where the anus remains open at rest. They discuss how sphincters become overstretched through transanal approach, deep dissection in the wrong plane, and retractor placement in the anus. Ahmad identifies a patulous anus in image 3 as indicating poor potential for bowel control.
11:38Management of Poor Sphincter Function — Discussion of mechanical bowel management programs for patients with lost potential for bowel control. Levitt describes using 3D anorectal manometry to objectively assess sphincter function and introduces a new sphincter tightening technique published in JPS. The team discusses when to use rectal enemas versus Malone procedures.
14:26Rectal Prolapse as Iatrogenic Complication — Review of a severe case showing rectal prolapse after Hirschsprung's pull-through, which Levitt emphasizes should never occur if sphincters are preserved. The image shows both overstretched sphincters and lost dentate line. Fisher explains that intact sphincters normally retract prolapsed mucosa after bowel movements. Levitt recommends sphincter reconstruction plus Malone for such cases.
18:17Summary and Preview — Jensen summarizes the key teaching points: assess potential for bowel control by evaluating sphincter squeeze (concentric or not) and dentate line integrity in awake patients, ideally with 3D manometry. Patients with poor sphincters or inadequate dentate lines need mechanical emptying. Preview of part 2 focusing specifically on dentate line issues.

Key claims

1:41Post-pull-through Hirschsprung's patients present in two distinct patterns: obstructed patients with distention, enterocolitis, and failure to thrive versus soiling patients who poop constantly and are never distended — Mark Levitt
4:09If the pull-through is done correctly with no anatomic problems and preservation of the sphincter mechanism, most Hirschsprung's patients do extremely well — Mark Levitt
4:34There is no reason why any Hirschsprung patient should be obstructed or soiling if properly managed and investigated — Mark Levitt
5:07Pathology can change over time; cases exist where ganglion cells were present at pull-through but absent with hypertrophic nerves 7 years later when problems developed — Jason Fisher
5:47Pull-throughs can decompensate if patients do not have adequate medical management or sphincter management, potentially leading to nerve hypertrophy — Mark Levitt
6:47The most important question for any soiling patient is: what is the patient's potential for bowel control — Mark Levitt
7:09All Hirschsprung's patients have the best possible potential for bowel control because they were born with normal sphincters and intact dentate line with anal canal sensation — Mark Levitt
7:38Hirschsprung's patients with intact dentate line and intact sphincters have all the potential to have voluntary bowel movements and bowel control — Mark Levitt
7:52If the dentate line is lost because dissection was started too low, or sphincters were overstretched and don't contract well, patients may have lost their potential for bowel control — Mark Levitt
9:153D anorectal manometry can visualize whether the sphincter squeeze is concentric; one patient had good squeeze but no squeeze on the anterior side — Mark Levitt
10:48Sphincters become overstretched through transanal approach with deep dissection in the wrong plane and retractors placed in the anus — Mark Levitt
12:20Patients with poor potential for bowel control due to overstretched sphincters need mechanical emptying programs with enemas or antegrade options — Mark Levitt
12:39Mechanical bowel programs can get borderline patients to a point where they are clean and psychologically want to be clean, making them more likely to successfully potty train — Mark Levitt
13:11Routine practice now includes 3D anorectal manometry in all soiling Hirschsprung's patients to assess sphincter squeeze and determine potential for bowel control — Mark Levitt
14:02A new sphincter tightening technique has been developed and published in JPS with great results in multiple patients with disrupted or patulous sphincters — Mark Levitt
15:02Rectal prolapse after Hirschsprung's pull-through is iatrogenic and should never occur if sphincters are preserved — Mark Levitt
15:26Overstretched sphincters become patulous to the point of prolapse, resembling a spina bifida anus — Mark Levitt
16:07During normal bowel movements, sphincters relax and some mucosa comes down, but as soon as sphincters tighten back up, mucosa is retracted back in; prolapse at rest indicates damaged sphincters — Jason Fisher
17:13For patients with patulous sphincters, sphincter reconstruction should be offered and can be done at the same time as Malone procedure — Mark Levitt
17:53Malone can serve as a bridge to continence by allowing patients to practice holding in the flush and releasing on command, potentially improving sphincter function to achieve bowel control — Mark Levitt

Points of disagreement

5:47Whether vanishing ganglion cells exist or represent original pathology error
- Mark Levitt: Pull-throughs can decompensate and nerves can hypertrophy due to inadequate management, but if there are no ganglion cells seven years later there probably weren't ganglion cells originally; the concept of vanishing ganglion cells related to tension or ischemia is quite controversial
- Jason Fisher: Cases exist where ganglion cells were documented present at pull-through but absent years later, suggesting pathology can change over time

Open questions

Whether ganglion cells can truly vanish over time due to tension or ischemia, or whether their absence years after pull-through represents original pathology error
The optimal timing and patient selection criteria for transitioning from mechanical bowel programs to attempted voluntary bowel control
Whether sphincter reconstruction combined with Malone will reliably restore continence in patients with iatrogenic sphincter injury

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:

Managing the Soiling Hirschsprung's Patient: Assessing Potential for Bowel Control

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 the care team · Explainer · AI-written, human-reviewed

Why This Problem Exists

Hirschsprung's disease requires surgical correction — a pull-through procedure that brings normally innervated bowel down to the anus. When the operation goes well, most patients do fine with minor medical management. But a subset presents with persistent soiling despite adequate emptying. These patients are never distended, never obstructed, and never fail to thrive. They simply stool constantly 1:41. This is a fundamentally different problem from the obstructed post-pull-through patient and requires a different diagnostic framework.

The Core Clinical Question

Before treating any soiling Hirschsprung's patient, one question determines the entire management path: what is the patient's potential for bowel control 6:47? This question is often neglected, yet it separates patients who need behavioral and medical management from those who need mechanical emptying programs.

Hirschsprung's patients start with an inherent advantage. Unlike children with anorectal malformations — who may have congenital spinal, sacral, or sphincter abnormalities — Hirschsprung's patients are born with completely normal sphincters and an intact dentate line 7:09. If those structures remain intact after surgery, the patient has full potential for voluntary bowel movements and continence 7:38. If the dentate line is lost because dissection started too low, or if the sphincters are overstretched and no longer contract effectively, that potential may be gone 7:52.

How to Assess Sphincter Integrity

The assessment begins with direct visualization of the awake patient. Normal sphincters keep the anus completely closed at rest. Patulous sphincters — those that have been overstretched — remain open, often with a visible slit or gap 10:48. This visual finding correlates with functional capacity.

Sphincters become damaged through specific technical errors during the transanal pull-through: deep dissection in the wrong plane and placement of retractors directly in the anus 10:48. Once overstretched, these sphincters do not recover.

Objective confirmation comes from 3D anorectal manometry, which can visualize whether the sphincter squeeze is concentric and quantify its strength 9:15 13:11. One patient had good overall squeeze but no anterior contraction — a finding that would be missed on standard examination 9:15. This technology is not universally available, but it has become routine practice in some centers for all soiling Hirschsprung's patients 13:11.

When Sphincters Are Damaged

Rectal prolapse after Hirschsprung's pull-through is a never-event if sphincters are preserved 15:02. Its presence indicates severe iatrogenic injury. During normal defecation, sphincters relax and some mucosa descends, but intact sphincters immediately retract it 16:07. Prolapse at rest means the sphincters cannot perform this basic function 15:26.

Patients with poor sphincter function need mechanical emptying programs — either rectal enemas or antegrade enemas via Malone appendicostomy 12:20. This is the same approach used for other conditions with poor bowel control potential, such as spina bifida or anorectal malformations with sacral abnormalities.

A new sphincter reconstruction technique has been developed for patients with disrupted or patulous sphincters, published recently in the Journal of Pediatric Surgery with promising early results 14:02. The procedure can be performed at the same time as Malone placement 17:13. Even when sphincter reconstruction is offered, mechanical emptying remains the foundation of management because the functional outcome cannot be guaranteed.

The Role of Mechanical Programs

Mechanical bowel programs do more than manage symptoms. For borderline patients, achieving consistent cleanliness creates the psychological foundation for successful toilet training 12:39. The Malone procedure can serve as a bridge to continence: patients practice holding the flush and releasing it on command, potentially improving sphincter function to the point of achieving voluntary bowel control 17:53.

When Practice Decompensates

Some patients have documented ganglion cells at the time of pull-through but develop aganglionosis with hypertrophic nerves years later when problems emerge 5:07. Whether this represents missed pathology at the original operation or true deterioration remains unclear. Pull-throughs can decompensate if patients lack adequate medical management or sphincter support, potentially leading to nerve hypertrophy 5:47. This underscores the importance of long-term follow-up and aggressive management of any constipation.

When to Involve This Team

Any Hirschsprung's patient with persistent soiling after pull-through warrants referral to a center with colorectal expertise. The evaluation should include assessment of sphincter integrity (visual examination and ideally 3D manometry) and dentate line preservation. Patients with intact anatomy need medical optimization and behavioral management. Patients with damaged sphincters or lost dentate lines need mechanical emptying programs, and some may benefit from sphincter reconstruction. The key teaching: there is no reason any Hirschsprung's patient should be soiling if properly investigated and managed 4:34.

Takeaways from this story

Ask first: does this patient have potential for bowel control? Intact sphincters and dentate line mean yes; damaged anatomy means no.
Visual exam of the awake patient reveals sphincter integrity: normal sphincters keep the anus closed; patulous sphincters show a gap or slit.
Rectal prolapse after Hirschsprung's pull-through is iatrogenic and indicates severe sphincter injury that will not recover spontaneously.
Patients with poor sphincter function need mechanical emptying programs; sphincter reconstruction is now available and can be done with Malone.
3D anorectal manometry objectively confirms sphincter function and can detect asymmetric defects missed on physical exam.

Assessing Sphincter Potential in the Soiling Hirschsprung's Patient

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 trainees · Teaching arc · AI-written, human-reviewed

The Fundamental Question That Shapes Everything

Post-pull-through Hirschsprung's patients present in two distinct patterns: obstructed patients with distention, enterocolitis, and failure to thrive versus soiling patients who are never distended but have constant stooling 1:41. For the soiling patient, the most important question—often neglected—is: what is the patient's potential for bowel control 6:47. This question determines whether you pursue medical management and potty training or commit to a mechanical emptying program. Getting this assessment wrong wastes months of family effort on an approach that cannot succeed.

The Hirschsprung's Advantage

All Hirschsprung's patients have the best possible potential for bowel control because they were born with normal sphincters and intact dentate line with anal canal sensation 7:09. Unlike anorectal malformation patients who may have congenital spine, sacrum, or sphincter abnormalities, Hirschsprung's patients start with intact anatomy. If the dentate line and sphincters remain intact after pull-through, they have all the potential for voluntary bowel movements and bowel control 7:38. This is the baseline assumption—unless iatrogenic injury has occurred.

Recognizing Sphincter Injury

Sphincter integrity can be assessed by examining the awake patient. Normal sphincters keep the anus completely closed at rest. If you can see into the anus—a visible slit or opening—the sphincters have been overstretched and may have lost function. Sphincters become overstretched through transanal approach with deep dissection in the wrong plane and retractors placed in the anus 10:48. These injuries are preventable with proper technique but may be irreversible once they occur.

If the dentate line is lost because dissection was started too low, or sphincters were overstretched and don't contract well, patients may have lost their potential for bowel control 7:52. 3D anorectal manometry can visualize whether the sphincter squeeze is concentric; one patient had good squeeze but no squeeze on the anterior side 9:15. Routine practice now includes 3D anorectal manometry in all soiling Hirschsprung's patients to assess sphincter squeeze and determine potential for bowel control 13:11.

The Never-Event

Rectal prolapse after Hirschsprung's pull-through is iatrogenic and should never occur if sphincters are preserved 15:02. Overstretched sphincters become patulous to the point of prolapse, resembling a spina bifida anus 15:26. During normal bowel movements, sphincters relax and some mucosa comes down, but as soon as sphincters tighten back up, mucosa is retracted back in; prolapse at rest indicates damaged sphincters 16:07. If you see prolapse in a post-pull-through patient, you are looking at severe sphincter injury.

Treatment Based on Potential

Patients with poor potential for bowel control due to overstretched sphincters need mechanical emptying programs with enemas or antegrade options 12:20. This is not a temporary measure—it may be permanent. However, mechanical bowel programs can get borderline patients to a point where they are clean and psychologically want to be clean, making them more likely to successfully potty train 12:39. For patients with patulous sphincters, sphincter reconstruction should be offered and can be done at the same time as Malone procedure 17:13.

The Bridge Concept

Malone can serve as a bridge to continence by allowing patients to practice holding in the flush and releasing on command, potentially improving sphincter function to achieve bowel control 17:53. This reframes mechanical management from permanent accommodation to active rehabilitation. A new sphincter tightening technique has been developed and published in JPS with great results in multiple patients with disrupted or patulous sphincters 14:02. The key insight: even after sphincter injury, function may be recoverable—but only if you correctly assess the starting point and choose interventions that match the patient's actual anatomy rather than their original diagnosis.

Takeaways from this story

Always ask what the patient's potential for bowel control is before treating soiling—this determines whether you pursue potty training or mechanical emptying.
Hirschsprung's patients born with intact sphincters and dentate line have full potential for voluntary control unless iatrogenic injury occurs during surgery.
Sphincters become overstretched through transanal approach with deep dissection in wrong plane and retractors placed in anus—these injuries are preventable.
Rectal prolapse after pull-through should never occur with preserved sphincters; its presence indicates severe iatrogenic sphincter injury requiring reconstruction.
Malone can serve as bridge to continence by allowing practice holding and releasing flush on command, potentially improving sphincter function to achieve control.

Vanishing Ganglion Cells in Hirschsprung Disease: Pathology Error or True Decompensation

The points where the speakers disagreed, with each position presented side by side. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Points of disagreement · AI-written, human-reviewed

The Clinical Scenario

A child undergoes pull-through for Hirschsprung disease 5:07. Pathology at the time of surgery documents ganglion cells at the anastomosis 5:07. Seven years later, the child develops obstructive symptoms 5:07. Repeat biopsy shows no ganglion cells and hypertrophic nerves 5:07. What happened?

The Decompensation Hypothesis

Mark Levitt acknowledges that pull-throughs can deteriorate over time 5:47. He proposes that inadequate postoperative management — specifically failure to maintain adequate flow and sphincter function — can lead to progressive changes in the bowel, potentially to the point where "it can even go to the point of hypertrophying the nerves" 5:47. In this framework, the original pathology was correct, but the pull-through decompensated because "the patient did not have adequate medical management or sphincter management" 5:47.

However, Levitt expresses fundamental skepticism about the concept of truly vanishing ganglion cells 5:07. When presented with cases where ganglion cells were documented at pull-through but absent years later, he states: "that's a whole another discussion" and immediately offers an alternative explanation — "the original pathology was off" 5:07. His position is that if ganglion cells are absent years after surgery, they probably were not present originally, despite what the pathology report said 5:07. The idea that ganglion cells could vanish due to tension, ischemia, or other mechanical factors remains, in his view, "quite controversial" 5:07.

The Pathology-Can-Change Position

Jason Fisher presents documented cases that challenge this interpretation 5:07. He describes reviewing pathology from a patient where "ganglion cells were present" at the time of pull-through, but years later when problems developed, biopsy showed "no ganglion cells and hypertrophic nerves" 5:07. Fisher frames this as evidence that the tissue itself underwent transformation, not that the original pathologist made an error 5:07.

This position does not specify the mechanism by which ganglion cells might disappear, but it accepts the documented pathology at both time points as accurate and concludes that something happened to the bowel in the intervening years 5:07. Fisher acknowledges that "some of it's out of our hands, pathology" 5:07, suggesting that factors beyond surgical technique may influence long-term outcomes.

Where They Agree

Both surgeons agree that pull-throughs can deteriorate over time 5:47. Both accept that inadequate postoperative management — failure to maintain regular stooling, failure to address constipation, failure to support sphincter function — can lead to progressive problems 5:47. Neither disputes that hypertrophic nerves can develop in a previously functioning pull-through 5:47.

They also agree on the practical management implications: when a patient develops obstructive symptoms years after pull-through, repeat biopsy is indicated, and the findings will guide further intervention regardless of what the original pathology showed 5:07 5:47.

What Would Resolve It

The discussants do not propose a study or evidence that would definitively settle whether ganglion cells can truly vanish. Resolving this would require serial biopsies from the same anatomic location in patients with deteriorating pull-throughs, with rigorous pathology review by multiple expert pathologists at each time point, ideally with immunohistochemistry for ganglion cell markers. Even then, sampling error — the possibility that the second biopsy simply missed ganglion cells that are still present but sparse — would remain a confounding factor.

The disagreement may ultimately be unresolvable with current methods, but it has limited clinical impact: both surgeons agree that patients with deteriorating pull-throughs need investigation and that inadequate postoperative management contributes to poor outcomes 5:07 5:47. Whether the mechanism involves true loss of ganglion cells or unmasking of originally inadequate resection, the management principles remain the same.

Clinical Implications

This disagreement matters most for how surgeons counsel families and interpret pathology reports 5:07 5:47. If ganglion cells can truly vanish, then even a technically perfect operation with confirmed adequate resection carries some risk of late failure through mechanisms beyond the surgeon's control. If vanishing ganglion cells do not exist, then late obstructive symptoms always indicate either original pathology error or inadequate postoperative management — both potentially preventable 5:07 5:47.

The truth likely lies somewhere between: some cases represent original pathology error, some represent true tissue changes over time, and distinguishing between them in individual patients remains difficult 5:07.

Takeaways from this story

Pull-throughs can decompensate years later due to inadequate flow management, potentially causing nerve hypertrophy even with initially correct pathology
Documented cases exist where ganglion cells present at pull-through are absent years later, though whether this represents pathology error or true tissue change remains controversial
Both positions agree that inadequate postoperative sphincter and bowel management contributes to pull-through deterioration regardless of mechanism

Topic overview

This discussion addresses post-pull-through Hirschsprung's disease patients who present with soiling rather than obstruction. The core clinical teaching is that all Hirschsprung's patients have the potential for normal bowel control because they are born with intact sphincters and dentate lines, but iatrogenic injury during surgery—specifically sphincter overstretching from transanal dissection or starting the dissection too low and losing the dentate line—can eliminate this potential. When sphincters are patulous or the dentate line is lost, patients require mechanical bowel management programs (enemas or Malone) rather than medical management alone. The discussants emphasize that the first question in any soiling Hirschsprung's patient is: what is the patient's potential for bowel control?

Key takeaways

All Hirschsprung patients are born with normal sphincters and dentate lines, giving them potential for voluntary bowel control. (7:09)
Iatrogenic sphincter injury from transanal dissection or low dissection losing the dentate line eliminates continence potential. (7:52)
Soiling patients with patulous sphincters need mechanical bowel programs (enemas/Malone), not medical management alone. (12:20)
3D anorectal manometry is now routine to assess sphincter function and guide management in all soiling Hirschsprung patients. (9:15)
Sphincter reconstruction can restore continence in patients with iatrogenic injury and should be offered with Malone if needed. (14:02)

Keywords

Hirschsprung's disease enterocolitis failure to thrive constipation soiling rectal prolapse anorectal malformation spina bifida pull-through transanal approach 3D anorectal manometry endorectal ultrasound exam under anesthesia Malone procedure rectal enemas

Hashtags

#HirschsprungDisease #PediatricSurgery #ColorectalSurgery #PostOpCare

Transcript

Click "Show Transcript" to view the full text (17825 characters)

Hey there listeners, it's Amanda Jensen from Riley Children's. Today we bring to you a very special episode of our colorectal quiz. It is episode 22 and we are gonna talk about Hirschsprung's disease again and specifically the soiling patient. Doctor Fisher, why don't you start us off? Welcome back, everyone. Here we are with the colorectal quizzes. Wow. We have been doing this for a long time, Mark. I feel like we might be on like season 2 almost. Fantastic. That was Doctor Jason Fisher from Cincinnati Children's. Yes, and, and the good news about that is you can, you know, skip ahead to the next, the next one, you can watch them in sequence, you can watch them out of order. It's not like the old days where you'd have to wait until the next week. That was Doctor Mark Levitt from Children's National. Although I'm sure there's some people out there that are uh listening to this in sequence live. I mean, literally live within the next week of it being created, but. What are we, uh, what are we talking about today? Last week was a good session on, on Hirschprung's disease, the obstructed patient. Seems like it needs a follow-up. Uh, you know, that's amazing. What a lead-in. I think that's why we've gotten out of the pilot series, and they actually rehired us for a second season. Because go figure, but the next topic is Hirschrung's disease, post pull through, not doing well due to soiling, not obstruction, but the opposite, too much coming out. How do we deal with it? Such problems. So, yes, so, uh, I actually like to talk about the problematic post- pull through Hirschprung's patient in two versions. The obstructed patient, they are not emptying, they're having distention, they're having enterocolitis episodes, multiple, they're having failure to thrive. That's what we talked about last podcast. Then there's a group of patients. That don't have any of those obstructive symptoms, or if they do, they're fairly minimal, they're basically some constipation. But they are soiling, they're pooping all the time, never distended. That's the patient that we're gonna talk about today. So remember, there's the soilers, and then there's those that are obstructed, and today we're gonna talk about soiling. Hey, Mark, let me ask you a question. We've done a few podcasts on Hirschsprung's disease, but I don't think we touched on this. And I think it's important because uh both of us have seen many patients with Hirschsprung's disease, and, When you meet a family, day of life 123, when the diagnosis is being constructed, and we finally get to a diagnosis of Hirschwrung's disease, what do you tell the family, the parents, what to expect long term? Because I think this is what frustrates some families, is that they're told. We're gonna do an operation. It's a somewhat complex operation, but after that, everything's gonna be fine. But yet, we're dedicating podcasts to things not being fine. So, what do you tell them? Yeah, this is a, you know, I think if you ask most surgeons, they would say Hirschrun's is a great operation because You know what to do, you get the pathology right, you do a nice surgery, pull through works, everything's great, you're done. And the reality is that's just simply not true for Hirschrun's disease, but I will tell you, it is definitely different than an anorectal malformation scenario, where, That perhaps is more frustrating, cause in that case, you can do the most perfect anatomic reconstruction, and because the patient has a problematic spine or sacrum, sphincter muscles, etc. you don't have a good result, you still have a soiling patient that you need to deal with. I believe that in Hirschprung's disease, if you get the pull through right, with no anatomic problems, and a preservation of the sphincter mechanism. Most of those patients do extremely well. Some of them need a little medical treatment, usually to tinker with constipation. Yeah. So that's what I tell them. If I am doing that operation, I'm very confident I can achieve that. Of course, you need nursing help, you need tinkering, you need to make sure that they're pooping once or twice a day. You need to help them get to the right consistency of stool, but there is no reason why any Hirschprung patient should be obstructed, and there's no reason why any Hirschprung patients should be soiling, and if they are, We need to investigate why, and we can fix them, and that's what we're gonna talk about today. I think that's the key is, I, I, I tell the families that there is the potential of issues. I mean, some of it's out of our hands, pathology, let's say, right? Uh, we all have good pathologists and, and we have to trust your pathology team, but sometimes, We have pathology that changes. I mean, we've had cases, Mark, I remember when we were working together, where there's a case where we reviewed the pathology, and at the time of pull-through, ganglion cells were present, and at the time of problems, 7 years later, we do a biopsy, and there's no ganglion cells and hypertrophic nerves. Oh, you know, well, that's a whole another, that's a whole another discussion, which I think we touched on a little bit, uh, last time that something about that pull through deteriorated. Or the original pathology was off. Right. And I'm not sure which it is, but I definitely concede that the pathology might have been perfect, and then that pulter decompensated, and why did it decompensate? Probably because the patient did not have adequate medical management or sphincter management. Which we're gonna get to, but basically, they need to flow well after their pull-through, and if they don't, and they don't get treatment for that, their pull-through can decompensate, and I think it can even go to the point of hypertrophying the nerves. I think that there are no ganglion cells seven years later, there probably weren't ganglion cells originally, but there is this concept of the vanishing ganglion cells related to tension or ischemia or whatever, which is quite controversial. Right. All right, I just want to dial in specifically on this Hirschprung's disease patient that is soiling. What is the most important question to ask? I'm gonna really emphasize this, to always ask the question, and by the way, this is true for any soiling patient. What is the patient's potential for bowel control? This is a question that is often neglected. There are patients that are soiling, that are getting treated. Without asking that question. And when it comes to Hirschprung's disease, as I said earlier, all patients have the best possible potential for bowel control. Why? Cause they were born with normal sphincters. And let me put a footnote there, sometimes too strong, where they don't relax, but they certainly aren't lax sphincters, and an intact dentate line anal canal sensation should be intact. So, when you take care of a Hirschrung's patient, and their dentate line is intact, and their sphincters are intact, they have all the potential to have voluntary bowel movements and to have bowel control. If their dentate line is lost, because the dissection was started too low, or their sphincters were overstretched. And don't swing back well and don't contract well, now they may have lost their potential for bowel control. So that is the number one question, and we're gonna go through a series of pictures. Again, I encourage you to get the app, because then you'll be able to see the pictures, and I'm gonna show you guys photos. I don't know if you've seen these before, and my question will be, how are the sphincters, or how is the dentate line? All right, so let's do the first picture. Yeah, again, very important, I think, to really grasp this part of the, the, this podcast is to get the stay current app and see these pictures because it really helps with the whole understanding of what's going on. So, getting the app is key here. All right, so the first picture. Is what I consider a very normal set of sphincters. If you're on the stay current app, you can click on image number one. It is, the anus is closed. These are all pictures with the patient awake, by the way. So that's what you want. Now, to formally check, you can do an anorectal manometry. I'm not sure what you guys are doing there, Jason in Cincinnati, but we are now doing 3D. Anorectal manometry, which is really, really cool, and you can see whether the sphincter is, uh, whether the patient has a good squeeze and whether that squeeze is, is concentric. Recently, we saw a patient that had a good squeeze, but there was no squeeze on the anterior side. Um, I, I think these are all adjuncts that are great, whether it be 3D, anorectal manometry, endorectal ultrasound, all looking at where is the muscle and using it on patients with Hirschpring's disease, post pull through or anorectal malformations, both, I think, great adjuncts to looking. But you made a subtle hint that these are all patients awake. Yes. And I think that's key to understanding versus a patient that you might do an exam under anesthesia, a little bit harder to assess the sphincters per se. Yeah, so, um, the sphincters, I, I, I like to get a look at how they look. Do they look patches, or do they look closed? And anorectal manometry is obviously an objective way of seeing whether they're intact or not. You can actually get the patient to squeeze for you. Alright, now go ahead and look at image #2 on the stay current app. And this is a picture, and if you look closely here, you see that you can actually see in to the anus. There's like a slit, and it's not closed. So, to me, this is a set of sphincters that may have been overstretched. And how do they get overstretched? Transiental approach, deep dissection, wrong plane, retractors in the anus. Very bad, the sphincters won't come back to normal. All right, now turn your attention to image number 3 on the state current app. Kira, what do you think? What say you on, on that photo? So this is the patient that's still awake. Uh, we can see that the anal opening is not completely closed. It's pattuous. The squeeze is not that great. I don't, um, it doesn't look like they're even trying to squeeze in this picture, but either way, the anus is not completely closed in this picture. So, as Doctor Levitt mentioned before. Either the dissection was started too low, there's some potential damage to the sphincters, and patients not able to um squeeze them. That was Doctor Hira Ahmad from Seattle Children's. So, our first question that we posed is what is the patient's potential for achieving bowel control with overstretched sphincters that cannot close? What would be your answer to that question in this patient? In this patient, I would suspect it would be unfortunately poor because they're not able to even have the anus closed in the resting position. Right, and for other patient groups that don't have good potential for bowel control, like ARM with a poor spine and a poor sacrum, like spina bifida. What do we do for the soiling patient with poor potential for bowel control? Yeah, so they generally need to be in a dedicated bowel management program, either on enemas, could potentially considered an anti-grade option. So Doctor Levitt, what's the bottom line here? They need a mechanical emptying if they have lost their potential for bowel control, and of course, there are patients that are borderline, but I think a mechanical program can get them to a point where they are clean. And psychologically want to be clean, and then more likely to successfully potty train, but in a patient with injured sphincters or lost dentate line or both, they need a mechanical program. Before starting a mechanical program, would you do an anorectal manometry in these patients? Yeah, so my routine now is to do a 3D anorectal manometry in all of these patients, and if they have a good squeeze, Then, you know that they have potential for bowel control. I note whether they have a dentate line or not, which we're about to discuss in the next little segment here. And then you can make a judgment call, whether you wanna try to potty train them with medicines like laxatives, or give them a break, and get them mechanically clean, and then try the laxatives another time. And whether you do rectal enemas or a Malone is another philosophical discussion that we can have on another podcast. Depends on how willing they are to tolerate rectal enemas, it depends on their age, but the bottom line is they need, I think they need a mechanical program to start, and then some of them need a mechanical program forever, and then some of them can transition over to normal bowel control. Nowadays, I do a 3D anorectal manometry. Because I've started to do a new technique, which we just wrote up in JPS of tightening of the sphincters, um, which has actually had great results. We've done it in a number of patients, and, and for a patient who had disrupted sphincters or pattula sphincters, tightening them up will, will be, um, I think will be ultimately a helpful, a helpful maneuver. All right, now let's look at image number 4. If you're on the stay current app, go ahead and click on that picture. And what do you see here? Um, so in this patient has a circumferential about 1 centimeter, um, 0.5 centimeter to 1 centimeter of circumferential rectal prolapse. Rectal prolapse after a Hirschrung's operation? Kira, what are you talking about? It's probably secondary to uh sphincters being damaged and then um them not being able to, or them, well, I guess they're not really squeezing that hard though, right? So no, no, no, this is, this is iatrogenic. There is no, there is no patient, you have never seen. And all those wonderful colorectal people that you have gotten the opportunity to train with, none of those people have ever had a rectal prolapse after Hirschsprung's pull-through, ever. Jason Fisher, never. Me, never. Why? Because the sphincters are preserved. If you overstretch the sphincter, you, this is one of the worst pictures I've ever seen. If you overstretch the sphincter, it will become pattuous. It almost looks like a spina bifida anus at this point. And then the additional problem here, in this particular picture, there's also no dentate line. So the sphincters have been overstretched to the point of laxity, to the point of prolapse, and the dentate line is also lost because the surgeon started the operation too low, and sewed the colon to the, to the anus, which we're gonna get to in the next series of of, of the photos. Harry, you were, you were dead on, I mean. The muscles help control prolapse, right? When everyone has a bowel movement, your, your sphincters relax, the anus opens up, some mucosa comes down, but as soon as you tighten back up, that is retracted back in. This is a patient at rest with prolapsed mucosa. The two things went awry. One, the, the muscles were damaged from stretch, from iatrogenic injury, a combination of both, and also, it looks like the mucosa was brought too low on their anastomosis, and so, That's an unfortunate picture. So in all, in all of these patients, they, I believe they need a mechanical program. Uh, we can visually see that these sphincters are problematic. I would like to objectively confirm that. I think the gold standard for that is 3D manometry. It's not available to everybody. But just looking at it, patula sphincters is a patient that needs a mechanical program, either enemas or uh from below, or, or enemas from above with a malone. Nowadays, if I found a patula sphincter, I would offer the patient a sphincter reconstruction, and I, um, I hope my colleagues around the world will learn how to do this because it's not not hard at all, it's uh technically good. Uh, to a reproducible operation, and Jason, I can show you a nice video of this, um, and as I said, it is written up in the technique section of JPS a couple of months ago. And if you're in the stay current app, go ahead and click on the link and it will bring you to the article that Doctor Levitt is referring to. That's what I would offer them, and I would do them alone at the same time. Because I don't know for sure that the sphincter reconstruction is gonna be the ticket, but I'm hoping that they will then be clean with the Malone, and actually the malone, as we talked about in another podcast, can be a bridge to continents, they can practice holding in the flush, releasing the flush on command, and maybe improve their sphincters, and actually get to the point of bowel control. All right, and that wraps up part one of a two-part series in the colorectal quiz, episode 22, Hirschrung's Disease and the soiling Patient. So to summarize for this episode, In a patient with Hirschsprung's disease that's not doing well, and specifically having issues with soiling, the most important first question is what is the patient's potential for achieving bowel control? And really to assess this, you want to assess both the dentate line and the sphincter. Regarding the sphincters, we want to know what is their squeeze and is it concentric regarding the dentate line. We want to know if it is intact. It's important to evaluate these things with the patient awake. In addition, a great way to analyze this is also through 3D anorectal manometry. Overall, if you think that either #1, the sphincter is poor, or #2, the dentate line is inadequate and this patient will have poor bowel control, really the answer and solution to this problem is mechanical emptying, and this can be in the way of A uh Malone or retrograde enemas. And with the next episode, we will be talking about Hirschman's disease and the soiling patient and specifically talking about the dentate line. Until next time, this is Amanda Jensen at Riley Children's. And remember, knowledge should be free.

Comments

Loading comments...