Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1

Published: 2021-08-16

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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
Mark Levitt — guest
Rebecca Rentia — guest
Jason Fisher — guest
Hira Hammad — guest

Chapters

0:00Case introduction and initial presentation — Introduction of a 7-year-old with Hirschsprung disease presenting with distended abdomen and obstructive symptoms after pull-through at 10 days of life.
2:20Initial management and rectal exam findings — Discussion of first-line treatment with rectal irrigations (requiring sedation), physical exam findings of gas and foul-smelling liquid stool, and history of 2-3 similar episodes in preceding weeks including PICU admission.
6:22Differential diagnosis of post-pull-through obstruction — Comprehensive review of anatomic causes of pull-through failure: stricture, twist, Soave cuff, Duhamel spur, non-functional segment, with emphasis on knowing the original operation type.
8:22Enterocolitis pathophysiology and management principles — Detailed discussion of why Hirschsprung enterocolitis causes severe illness (fluid sequestration, bacterial overgrowth, translocation), importance of early recognition, and the critical role of rectal irrigation as 'CPR of the colon.'
11:47Diagnostic workup and contrast study interpretation — Review of enterocolitis scoring systems, timing of contrast enema after stabilization, and the importance of family irrigation training before discharge.
18:47Contrast enema findings and working diagnosis — Detailed description of contrast study showing dilated colon narrowing over distal 6 cm with widened presacral space, interpreted as either retained aganglionosis or constricting Soave cuff.

Key claims

11:06Hirschsprung enterocolitis can occur before surgery, after surgery, and even after successful surgery when patients do not relax their sphincters and hold stool efficiently — Mark Levitt
10:10In Hirschsprung enterocolitis, the colon fills with liquid stool with severe bacterial overgrowth, causing fluid loss into the bowel lumen, hypovolemia, and bacterial translocation leading to bacteremia, all with no passage of stool — Mark Levitt
14:08Post-pull-through enterocolitis within the first 3 months occurs in about 20% of cases — Mark Levitt
6:45Any type of pull-through can have a stricture or a twist regardless of the initial type — Hira Hammad
6:59Specific to Soave pull-through, one must consider Soave cuff as a cause of obstruction — Hira Hammad
7:06Specific to Duhamel pull-through, one must consider Duhamel spur or a large distended segment that is not functioning — Hira Hammad
7:15With Swenson pull-through, one could potentially have a stricture and a twist — Hira Hammad
7:22Rehbein procedure can present with a non-functional segment that does not empty — Hira Hammad
3:19The anatomy of the original pull-through could explain the patient's obstructive symptoms and whether there is a fixable problem — Mark Levitt
7:48A contrast study is helpful for inferring the original surgery type based on findings when the operative note is unavailable — Mark Levitt
8:25In a very ill child, a contrast study would not be the best initial option; resuscitation takes priority — Rebecca Rentia
16:40If sedation in the ER does not work for a severely ill patient, one should go to the OR under general anesthesia to irrigate until the child feels better — Mark Levitt
16:50In rare circumstances, an ileostomy may be needed to get the child out of trouble, with workup of the pull-through problem deferred — Mark Levitt
12:06If a patient with past Hirschsprung diagnosis comes in sick, one must assume Hirschsprung-associated enterocolitis; treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis turns out to be different — Jason Fisher
14:58Families should not undergo Hirschsprung pull-through surgery until they can demonstrate ability to perform irrigations — Jason Fisher
15:24Families should be discharged with supplies (Foley catheter, saline, syringe, bucket) to perform irrigations at home, as this could be life-saving — Jason Fisher
15:50Irrigation is the CPR of the colon for Hirschsprung patients — Rebecca Rentia
4:51When performing initial rectal exam on a distended Hirschsprung patient, the examiner should step to the side to avoid an explosive release of stool — Jason Fisher
19:14The presacral space (space between the hollow of the sacrum and the pull-through) is an important observation on contrast studies — Mark Levitt
19:46A lateral view of the rectum on contrast study provides important information and should be obtained — Jason Fisher
20:48On contrast enema, if the catheter balloon is over-inflated or inserted too high, distal pathology cannot be easily ascertained — Rebecca Rentia
21:40Widened presacral space on contrast study suggests a Soave pull-through — Jason Fisher
21:57A dilated colon narrowing over the distal 6 cm with widened presacral space suggests either retained aganglionosis or a long Soave cuff constricting the distal neorectum — Jason Fisher

Cases discussed

1:127-year-old with Hirschsprung disease presenting with obstructive enterocolitis 7 years after pull-through

Open questions

What was the original type of pull-through performed at 10 days of life?
Is the obstruction due to retained aganglionosis or a constricting Soave cuff?
What will the examination under anesthesia reveal about the anatomy?
Should biopsies be obtained during the examination under anesthesia?
How can the 20% rate of post-pull-through enterocolitis in the first 3 months be reduced?

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 Obstructed Post-Pull-Through Patient: A Systematic Approach

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

Recognize enterocolitis as an emergency requiring immediate intervention

When a patient with prior Hirschsprung surgery presents acutely ill, assume Hirschsprung-associated enterocolitis until proven otherwise 12:06. The pathophysiology explains the severity: the colon fills with liquid stool and severe bacterial overgrowth, causing fluid loss into the bowel lumen leading to hypovolemia, plus bacterial translocation causing bacteremia—all with no passage of stool 10:10. This is not ordinary gastroenteritis. The treatment triad of rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis turns out different 12:06, so act on the presumption rather than waiting for confirmation.

Master irrigation as the primary intervention

Irrigation breaks the cycle of stasis and is the definitive initial treatment. One discussant called it "CPR of the colon" [q1]—a phrase worth remembering because it captures the urgency. Families should never undergo pull-through surgery until they can demonstrate competence performing irrigations 14:58, and they must be discharged with supplies: Foley catheter, saline, syringe, and bucket 15:24. The first post-operative irrigation should be performed by someone who knows the anastomosis location 16:40. When performing an initial rectal exam on a distended patient, step to the side to avoid the explosive release of stool 4:51—practical advice that separates those who have done this from those who have not.

Know the mechanical differential by procedure type

The anatomy of the original pull-through determines what can go wrong 3:19. Any pull-through can develop a stricture or twist 6:45. Soave procedures risk a constricting cuff 6:59. Duhamel procedures can develop a spur or a large distended non-functional segment 7:06. Swenson procedures can present with stricture and twist 7:15. Rehbein procedures may leave a non-functional segment that does not empty 7:22. When the operative note is unavailable, a contrast study can help infer the original surgery type 7:48—but only if you know what to look for.

Read the contrast study with surgical eyes

Three observations matter most. First, assess the degree and length of proximal dilation versus the narrowed distal segment. Second, examine the presacral space—the distance between the hollow of the sacrum and the pull-through 19:14. A widened presacral space suggests a Soave pull-through 21:40. Third, evaluate study technique: the catheter balloon must not be over-inflated or inserted too high, or distal pathology cannot be ascertained 20:48. A lateral view of the rectum is essential 19:46. When you see a dilated colon narrowing over the distal 6 cm with widened presacral space, consider either retained aganglionosis or a long Soave cuff constricting the distal neorectum 21:57.

Escalate appropriately when initial measures fail

If sedation in the emergency room does not allow adequate irrigation in a severely ill patient, proceed to the operating room under general anesthesia and irrigate until the child improves 16:40. In rare circumstances, an ileostomy may be needed to stabilize the patient, with definitive workup of the pull-through problem deferred 16:50. Resuscitation takes priority over imaging in the very ill child 8:25.

Understand the timeline of post-operative enterocolitis

Enterocolitis can occur before surgery, after surgery, and even after successful surgery when patients do not relax their sphincters and hold stool efficiently 11:06. Post-pull-through enterocolitis within the first three months occurs in about 20% of cases 14:08—a significant rate that has proven difficult to reduce despite coordinated efforts. The discussants emphasized that families arriving at the emergency room with mild symptoms could have been managed quickly at home with irrigation [q2], underscoring why discharge preparation is not optional but life-saving [q5].

Takeaways from this story

Assume enterocolitis in any post-Hirschsprung patient presenting acutely ill; irrigation, fluids, and antibiotics won't harm if wrong
Families must demonstrate irrigation competence before pull-through surgery and go home with supplies—it's life-saving
Widened presacral space on lateral contrast view suggests Soave pull-through; proper catheter position is critical to see distal pathology
Each pull-through type has specific mechanical complications: Soave cuff, Duhamel spur, Swenson twist—know the differential
Post-pull-through enterocolitis occurs in 20% within first 3 months; can happen even after successful surgery if child holds stool

Post-Pull-Through Obstruction: Recognizing Anatomy, Resuscitating Aggressively, Reading the Contrast Study

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

Enterocolitis pathophysiology demands immediate intervention

The patient with Hirschsprung-associated enterocolitis is not simply dehydrated from diarrhea — they are obstructed 10:10. The colon fills with liquid stool and severe bacterial overgrowth, causing fluid loss into the bowel lumen and hypovolemia, while bacterial translocation produces bacteremia 10:10. All of this occurs with no passage of stool 10:10. The clinical picture is a distended, toxic child whose physiology is collapsing despite the absence of visible stool output. If a patient with a history of Hirschsprung disease presents sick, assume enterocolitis 12:06. Treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis turns out to be different 12:06. Resuscitation takes priority over imaging 8:25. In a very ill child, a contrast study is not the best initial option 8:25. If sedation in the ER does not work, go to the OR under general anesthesia and irrigate until the child improves 16:40. In rare circumstances, an ileostomy may be needed to get the child out of trouble, with workup of the pull-through problem deferred 16:50.

Irrigation is non-negotiable competency for families

Families should not undergo Hirschsprung pull-through surgery until they can demonstrate ability to perform irrigations 14:58. This is not a suggestion — it is a prerequisite. Families should be discharged with supplies: Foley catheter, saline, syringe, and bucket 15:24. This could be life-saving 15:24. "Irrigation is CPR of the colon" [q1]. The first irrigation post-operatively should be performed by someone who knows the anastomosis location, but thereafter the family must be autonomous. When performing the initial rectal exam on a distended Hirschsprung patient, step to the side to avoid an explosive release of stool 4:51. This is not merely practical advice — it reflects the degree of obstruction you are about to relieve.

The differential diagnosis is operation-specific

Any type of pull-through can have a stricture or a twist 6:45. Beyond that, the anatomy of the original pull-through could explain the patient's obstructive symptoms and whether there is a fixable problem 3:19. Specific to Soave pull-through, consider Soave cuff as a cause of obstruction 6:59. Specific to Duhamel pull-through, consider Duhamel spur or a large distended segment that is not functioning 7:06. With Swenson pull-through, one could potentially have a stricture and a twist 7:15. Rehbein procedure can present with a non-functional segment that does not empty 7:22. A contrast study is helpful for inferring the original surgery type based on findings when the operative note is unavailable 7:48. You must be an expert at reading these studies, and your radiologist must be trained in what to look for.

Contrast study interpretation depends on presacral space and technique quality

The presacral space — the space between the hollow of the sacrum and the pull-through — is an important observation on contrast studies 19:14. A lateral view of the rectum provides important information and should be obtained 19:46. Widened presacral space suggests a Soave pull-through 21:40. A dilated colon narrowing over the distal 6 cm with widened presacral space suggests either retained aganglionosis or a long Soave cuff constricting the distal neorectum 21:57. On contrast enema, if the catheter balloon is over-inflated or inserted too high, distal pathology cannot be easily ascertained 20:48. A good study requires proper catheter position, adequate seal, and appropriate balloon inflation. Without these, you are reading noise.

Post-pull-through enterocolitis is common and not yet preventable

Post-pull-through enterocolitis within the first 3 months occurs in about 20% of cases 14:08. Hirschsprung enterocolitis can occur before surgery, after surgery, and even after successful surgery when patients do not relax their sphincters and hold stool efficiently 11:06. Efforts to reduce the early post-operative rate have not yet succeeded. The implication is that vigilance and family preparedness are the only reliable defenses. Every family must leave the hospital knowing how to irrigate and equipped to do so.

Takeaways from this story

Enterocolitis physiology is obstruction plus bacterial overgrowth causing hypovolemia and bacteremia with no stool passage
Families must demonstrate irrigation competency before pull-through and go home with supplies—this is life-saving
Widened presacral space on lateral contrast view suggests Soave pull-through; narrowing over 6cm suggests cuff or aganglionosis
Post-pull-through enterocolitis occurs in 20% within 3 months; no prevention strategy has yet reduced this rate

Post-Pull-Through Obstruction Seven Years Later: Anatomy as Diagnosis

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Case narrative · AI-written, human-reviewed

Presentation

A seven-year-old presented to the emergency department with severe abdominal distension 11:06. The child appeared sick — tachycardic, dehydrated, potentially septic [case1]. History revealed Hirschsprung disease treated with pull-through at ten days of life at another institution; the operative note was unavailable, and the child had never been on rectal irrigations [case1]. On rectal examination, a large volume of gas released, followed by foul-smelling liquid stool — significant but not explosive — with harder stool palpable distally [case1]. The family reported two to three similar episodes over the preceding three to four weeks, one severe enough to require PICU admission elsewhere [case1].

Abdominal radiographs showed no free air or pneumatosis. Labs and physical exam suggested hypovolemia and possible sepsis. The working diagnosis was Hirschsprung-associated enterocolitis — a syndrome in which the colon fills with liquid stool and severe bacterial overgrowth, causing fluid loss into the bowel lumen, hypovolemia, and bacterial translocation leading to bacteremia, all with no passage of stool 10:10. This can occur before surgery, after surgery, or even after successful surgery when patients do not relax their sphincters efficiently 11:06.

The Decision Point

The immediate question was not whether to resuscitate — that was straightforward — but how to determine what had gone wrong seven years after an apparently successful pull-through. Without the original operative note, the team faced a diagnostic problem: was this functional obstruction from poor sphincter coordination, or was there a structural problem with the pull-through itself that could be corrected?

The anatomy of the original pull-through could explain the patient's obstructive symptoms and whether there was a fixable problem 3:19. Any pull-through can develop a stricture or a twist 6:45. Specific to Soave, one must consider a constricting Soave cuff 6:59. Specific to Duhamel, a Duhamel spur or a large distended non-functioning segment 7:06. Swenson can present with stricture and twist 7:15; Rehbein with a non-functional segment that does not empty 7:22. The team needed to identify the original procedure and assess for these complications.

In a very ill child, a contrast study would not be the best initial option; resuscitation takes priority 8:25. The child was admitted for IV antibiotics and sedated rectal irrigations three times daily. After significant improvement with these initial measures, the team proceeded to contrast enema performed in the operating room during examination under anesthesia [case1].

What They Did

The contrast study showed a dilated colon narrowing over the distal six centimeters, with widened presacral space between the pull-through and the sacrum [case1]. A lateral view of the rectum provided critical information 19:46. The catheter was positioned correctly — not over-inflated or inserted too high, which would have obscured distal pathology 20:48.

The widened presacral space suggested a Soave pull-through 21:40. The pattern of dilation narrowing over the distal six centimeters with widened presacral space suggested either retained aganglionosis or a long Soave cuff constricting the distal neorectum 21:57. When the operative note is unavailable, a contrast study is helpful for inferring the original surgery type based on findings 7:48.

The working diagnosis became an anatomic or pathologic issue in the distal six centimeters of the pull-through — either retained aganglionosis or a constricting Soave cuff [case1].

Outcome

The child improved significantly with initial resuscitation measures [case1]. Further management — whether to proceed with biopsy to distinguish retained aganglionosis from mechanical obstruction, and what surgical revision if any would be required — was deferred to subsequent discussion. The outcome beyond stabilization was not discussed [case1].

What This Case Changes

Post-pull-through enterocolitis within the first three months occurs in about 20% of cases 14:08, but this child presented seven years later, having apparently done well in the interim. The case underscores that families should not undergo pull-through surgery until they can demonstrate ability to perform irrigations 14:58, and should be discharged with supplies — Foley catheter, saline, syringe, bucket — to perform irrigations at home, as this could be life-saving 15:24. If a patient with past Hirschsprung diagnosis presents sick, one must assume Hirschsprung-associated enterocolitis; treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis turns out to be different 12:06. If sedation in the ER does not work for a severely ill patient, one should go to the OR under general anesthesia to irrigate until the child feels better 16:40; in rare circumstances, an ileostomy may be needed to get the child out of trouble, with workup of the pull-through problem deferred 16:50.

The transferable judgment is this: when a post-pull-through patient presents with obstruction, the original anatomy matters. A contrast study interpreted by someone who understands pull-through variants can distinguish functional from structural problems and guide whether surgical revision is indicated.

Takeaways from this story

Post-pull-through enterocolitis can occur years after successful surgery when sphincter dysfunction causes stool retention.
Widened presacral space on lateral contrast view suggests Soave pull-through; narrowing over distal 6 cm suggests cuff or aganglionosis.
Families must demonstrate irrigation competence before pull-through and go home with supplies—it can be life-saving.
Assume enterocolitis in any post-Hirschsprung patient presenting sick; empiric treatment causes no harm if diagnosis differs.

Topic overview

A multidisciplinary discussion of a 7-year-old with Hirschsprung disease who presented with obstructive enterocolitis seven years after a pull-through of unknown type performed at 10 days of life. The panel reviews initial resuscitation with rectal irrigations, IV fluids, and antibiotics, emphasizing that irrigation is the cornerstone of breaking the obstructive cycle. Contrast enema revealed a dilated proximal colon narrowing over the distal 6 cm with widened presacral space, suggesting either retained aganglionosis or a constricting Soave cuff as the anatomic cause of obstruction.

Key takeaways

In obstructed post-pull-through Hirschsprung patients, knowing the original pull-through type (Swenson, Soave, or Duhamel) is critical to diagnosis.
Rectal irrigations are first-line for Hirschsprung enterocolitis, but may require sedation in older children; stand aside during exam for safety.
Foul-smelling stool with gas on rectal exam suggests enterocolitis; absence of palpable stricture doesn't rule out anatomic obstruction.
Recurrent enterocolitis episodes (especially requiring PICU admission) warrant anatomic evaluation, not just medical management.
In septic-appearing Hirschsprung patients, initiate IV antibiotics and sedated irrigations immediately while investigating underlying cause.

Keywords

Hirschsprung disease Hirschsprung-associated enterocolitis enterocolitis bacterial overgrowth bacteremia hypovolemia retained aganglionosis stricture Crohn's disease pull-through Swenson pull-through Soave pull-through Duhamel pull-through Rehbein procedure rectal irrigation

Hashtags

#HirschsprungDisease #PediatricSurgery #Enterocolitis #ColorectalQuiz

Transcript

Click "Show Transcript" to view the full text (22165 characters)

Hey there listeners, it's Amanda Jensen from Riley Children's. Have you downloaded the new version of the Stay Current app? It is in the Google Play Store and in the Apple App Store. The reason I bring it up is that if you are listening to this episode within the app, you can bring up the pictures and journal articles discussed in real time while we are talking about them. Today we have a very special episode for our colorectal quiz. I'll start off by introducing Doctor Mark Levitt from Children's National. All right, Mark, what are we talking about today? Troublemaker Hirschprung's, the patient with Hirschsprung's disease who is not doing well after their pull-through with obstructive symptoms, cause of course, there's a whole discussion about patients with stools that go too fast. Today, we're gonna be talking about obstructive symptoms. Rebecca, welcome. Hey, thanks so much for having me. Really excited about everything and talking on this podcast. That's Doctor Rebecca Rentia joining us from Children's Mercy in Kansas City. Why don't you start by telling us about your case? Yeah, so we have a seven year old child who presented, uh, for the first time to our emergency room with a history of Hirschsprung's and a, uh, pull-through of unknown type. That was performed at 10 days of life at a referring institution. And basically, they came, the child came in with a very distended abdomen, and that's really all that we knew when the child presented. So, it basically, in summary, you have a 7 year old child with known Hirschprung's, had a pull through, you don't know what type, you can't figure out what type. And they are behaving like they are obstructed, so much so that they're having the ultimate obstruction, and that is uh enterocolitis. Correct. It's an older child, which always presents some of the, the big questions that we'll talk about. Yeah, and so, um, there they are in your ER distended, looking sick, tachycardic, dehydrated, and potentially having some transitive bacteria and uh translocation, behaving septic. It's a problem. Signs and symptoms here that Doctor Levitt is describing are non-specific findings you see in Hirschprung's enterocolitis. First line of attack, first line of attack after an abdominal X-ray and labs and a good physical exam is to try rectal irrigations, which were not tolerated. Oh, so you didn't explain to the seven year old child that rectal irrigations were gonna, gonna make him feel better, and he immediately said, whatever you say, doc, thank you for helping me. Correct. So, basically, we have this child with a really distended abdomen, a trial of irrigations that ultimately to get any of the irrigation done, we had to sedate the child. It's a very important discussion. I, I, let's take it back a notch. If you have an obstructed patient who had a previous history of Hirschsprung's disease, we're sort of alluding to the fact it's awfully nice to know what Hirschprung's pull-through they had in the past. And I really want to emphasize this point, particularly to the gastroenterologist, that the anatomy of the pull-through done originally could very well explain the patient's symptoms. And we need to know what that anatomy is and whether there's a problem with that pull-through that we could ultimately fix and make this kid no longer behave obstructed. And the three main types of pull-throughs that Doctor Levitt is referring to here is the Swensen, the Suave, and the Duel. We'll go into further details later on in this podcast. All right, Rebecca, what was your next course of action? So our plan here for this child, one of the first things that we did was to admit them because we needed to figure out how to make them better. So, we initiated IV antibiotics. We started sedated irrigations. On rectal exam, there was no obvious stricture that could be felt, that could be the cause of this. But other than that, that was the first kind of steps. And so the child was admitted to getting 3 times a day, sedated irrigations, and then our next step was kind of what do you do with this slightly older child who's pretty old. Can we, can we step back a second? That's Doctor Jason Fisher from Cincinnati Children's. So on rectal, on the initial rectal exam. Or attempted, I guess in combination with the attempted irrigation. Did you get an explosive, what came out? What was the result of that first exam? Was there an explosion of stool? Cause I think one key thing to talk about with all our listeners is when you do that exam, make sure you step to the side. A little bit funny, but a little bit, you don't wanna be in the way of what could be coming out. So what came out on this child? What did you find? Clearly, a, a seven year old's hard to do irrigations on any child, especially we could talk about, and this wasn't your patient, did this child ever have symptoms like of enterocolitis before? Did they ever get irrigations before? Were they ever, was the family ever taught about this? So just curious, a little bit more in retrospect, what, what was there? So, um, so on examination, there was a large amount of gas that came out. Um, when the catheter was passed up and on digital exam, a significant but non-explosive amount of foul smelling stool that was liquid was. And then when the catheter was attempted to be passed to do a complete irrigation, you could feel that there was harder pieces of stool down below as well. So it was kind of a mix of all of the things that you might see, but definitely the foul smelling stool. And This child, after discussing more with the family, their past history, they've never been on irrigations, but prior to coming to our hospital about 3 to 4 weeks before that, they'd had 2 to 3 other significant similar episodes, one of which landed them in the PICU at, at that other hospital. Wow, yeah, so pretty significant past history. Let's talk about the possibilities here of what could be wrong with. This pull-through. So why don't you give me a list of the anatomic reasons why a pull-through is not working well for a patient. We also have with us today, Doctor Hira Hammad from Seattle Children's. Right, so every time we have a post- pull-through patient who's presenting with obstructive problems, you know, any type of pull-through can have a stricture or a twist. That's regardless of the initial type of pull-through they had. And then specific to the type of pull-through, um, you know, if they have a previous Suawe, we have to think about swaawe cuff. If they have a previous Duomel, then we have to think about a Duomel spur or just this large distended segment that's just not functioning. And then there's other types like with Swenson, you could potentially have a stricture, and as I mentioned, a twist as well. The other thing to think about is depending on when they have their pull-through, there's other types like Raine procedure, which could just present with this non-functional segment that just does not empty. So all of those are mechanical. Problems with the, with the pull through segment. Yeah, that's great. Those are, uh, that's a very complete list, and I think if you can get the original uh operative note, great. But if you can't, a contrast study is really helpful, and sometimes you can infer the original surgery based on the findings of the contrast study. So I think you really need to be an expert. At looking at these contrast studies and getting your radiologist totally in tune with how to look at contrast studies post pull-through. So, I suspect, Rebecca, after you settled this kid out with some sedation, some irrigation, got him in control, got him some IV fluids, Your next step was probably a contrast study. Am I right? Yup, correct. So we got nothing concerning on the X-ray. I think another important point is that a contrast study, while extremely valuable, um, in a, in a very, very ill child would not be the best option. But since They did improve significantly from our initial maneuvers and there was no large amount of speculation or some imprinting on the X-ray. We went ahead with the contrast study. Let, let's go back one second because I think we all just touched a little bit, but a really important. Subject in this situation is how sick these children can get. And we have, uh probably, I assume everyone on this call has seen a child really sick from Hirschprung's disease. You mentioned just before, a couple of weeks earlier, this child was in a PICU at a different institution. So these children can get really ill, and IV hydration, the rectal irrigation, starting systemic antibiotics, all important steps. I think we all agree the irrigation is extremely important, and then these children can be very behind. In their fluid resuscitation and making sure their resuscitation is good. In fact, we had a child get so sick a few years ago, we developed a protocol. We'll link it to, to our podcast, going over all these things cause you know, these things happen at 2 in the morning, they don't happen at 2 in the afternoon. If you're listening to the podcast in the stay current app, you can click on the link below to see the protocol. You know, I'm, I'm glad you mentioned that because the gastroenterologists and pediatricians on the call. I think it's important for them to understand why the patients get so sick. Now, of course, they get, um, they're used to patients with very bad diarrhea getting very dehydrated. But I think the same concept is happening here. They're getting diarrhea, but the poop is not coming out because they're obstructed. But their colon is filling with liquid stool with a severe amount of bacterial overgrowth, lots of loss of fluid into the lumen of the bowel, hypovolemia, and then, of course, translocation and bacteremia from that. All of that is happening with no passage of stool. However, of course, you see that the patient is distended. So I think when a pediatrician has someone in their practice that had Hirschsprung's disease, A little bit of diarrhea or certainly problematic distention, irritable child, fever with distention is enterocolitis until proven otherwise. And as you mentioned, you gotta break the cycle, you gotta stop the stasis, and irrigation is the, is the best way to go because they're not passing stool because of distal obstruction. And it's important to remember this can happen before surgery. It can happen after surgery, and in a baby, it can happen after successful surgery. And we all know that, we talked about that on another podcast. The patients don't relax their sphincters, they're not coordinating their sphincters, they can hold their stool in so efficiently that they also can develop enterocolitis. But this case at age 7 to me, sounds like we're gonna find something anatomically. Or pathologically, we didn't mention that wrong with this pull through. So, Rebecca, let's go over, let's take a look at the contrast study and see if we can learn anything from it. So before we move on, I know I've, I've always had this question, you know, how do you diagnose enterocolitis, and there's several criteria that are out there. Is there one that you guys prefer over the other, or as Doctor Levitt just mentioned, you know, clinically, if you have then you just start the treatment. Well, I'll start by saying, if you have a patient that comes in with a diagnosis of Hirschprung's in the past and comes with, comes in sick, you have to assume it's Hirschprung's associated enterocolitis. None of this treatment will injure or deflect or cause harm. And, if the diagnosis is different, let's say it turns out to be Crohn's disease or um just a E. coli, enterocolitis, or whatever it might be. If you give rectal irrigations, IV fluid resuscitation, and systemic antibiotics, you're not burning a bridge. Or causing any harm from a different diagnosis. And Jason, I'm gonna underscore that point. I think there's a lot of new literature that just came out talking about various protocols of treating enterocolitis at home or maybe having a um lesser length stay in the hospital, but I would say the underlying point is that there's either a really, really good System whereby children are known and evaluated and so they can have a treatment course at home versus they're in the hospital and there's a protocol to be able to address their needs so that it's basically it's better to overtreat than it is to miss this diagnosis. I think that's a fantastic point. You're dead. I, I also, you know, you have to be careful when you read the literature and they quote percentages of enterocolitis. What do they mean? Do they mean admission? Do they mean needed irrigations? Did they mean needed antibiotics? Um, there is a very nice score. Um, I know the PCPLC, the consortium is working on validating the Langer score of, um, HAEC so that we can really apply it uniformly. When you read articles about Hirschspring disease, just be aware. I will tell you, we did study, Jason, I'm sure you remember, post- pull-through enterocolitis in Cincinnati and in Columbus. Within the 1st 3 months was about 20%, which is a significant number. We, we put our, pulled our group together in all those cases, and we're trying very hard to reduce the rate, and it doesn't seem like we're succeeding in figuring out how to do that. In my personal practice, I make sure the families know how to do an irrigation. They have the supplies in order to do it. Obviously, you need to wait a week or two after the surgery and maybe the first irrigation should be performed by someone who actually feels confident, knows where the anastomosis is, etc. but families need to intervene. There's nothing worse, there's nothing worse than having a patient in an ER with mild symptoms that could have gotten the patient better very quickly. With an irrigation, I, I think, I think we're hitting a huge point that obviously is not this case that Rebecca is presenting because the patient was treated elsewhere and, and new to your, your institution and, and your program, but I think everyone on this podcast would say you do not do an operation on a Hirschprung's patient until the family can demonstrate that they are able to do irrigations. Um, beforehand. I know I don't, and, and it's a must, and we make sure they have the supplies, and on discharge, there's a whole coordinated effort to make sure they go home with a goodie bag of supplies to take care of doing an irrigation, cause, uh, let's be honest, it could, a simple thing of a Foley catheter, some saline, and a syringe, and a, and a bucket could be life-saving. So I think it's extremely important. Irrigation is CPR of the colon is how I compare it to family. I love it. Love it. All right, back to the case. Let's talk a little bit more about the contrast enema. The contrast study, for those of you who have the app. You can take a look at the contrast study, and I think it's very, very interesting, very instructive. So let me describe it to you for those of you who can't see the picture. Mark, just to reiterate, and I know we touched on this, it is not vital to get this contrast study on the initial presentation for this patient, right? We resuscitated the patient. Patient responded like Rebecca said to the resuscitation. And now we're digging for the diagnosis. Yeah, absolutely, and I would also say, just so you have all of the items you need in your armamentarium, if you have a patient that's so ill and sedation in the ER doesn't work, I would go to the OR, general anesthesia. Irrigate, irrigate, irrigate until they feel better. And there are some circumstances where you need to do an ileostomy, just to get the kid out of trouble and then work up what's wrong with the pull-through down the road. That's quite rare, but be prepared. So would you do a biopsy or consider doing a biopsy at the same time, or would you work up this patient later? Well, I would certainly, I, I mean, it's a good question. I would certainly do an exam under anesthesia. And let's talk about that. Wait, let's go back. Hold on. Are you asking at the time you go do the ileostomy or at the time of the workup of this patient? Yeah, no, at the time of the workup. So this patient is really sick. We're taking to the OR under sedation, and we do a ton of rectal irrigation, and then the fluid is clear. Jason, I, I knew what she was saying. I already speak here, um, after 3 years. I'm very fluent in Hira, um, but, um, the, uh, I mean, it's sort of a, we have to answer this in two ways. If we end up going to the operating room emergently. We're obviously gonna do a rectal exam. Um, we're gonna do irrigations, etc. Let's talk about that rectal exam in Rebecca's patient. She's gotten the patient better. Irrigations have worked, the kid's properly hydrated. Now it's time to figure out what's wrong with this pull-through. Contrast study, we're gonna talk about, let me describe that, and then from there, the next step here, uh, maybe you'll go over what exactly you're trying to feel on that rectal exam. What, what data are you trying to understand to make an explanation for why this pull-through isn't working. For those in a, in a logical workup, I would, if, if you're not forced to go to the OR emergently. I think we all agree we get the contrast study before going to the operating room for a rectal exam, which we know we're doing also because he might unravel something or, or give you some hints into what to do next. But let's talk about this contrast study. So I'm gonna, I'm gonna describe the contrast study, but I'm not gonna give you my impression of the contrast study. I'm just gonna say what I see for those of you to try to, while you're in your car or you can't open the, you don't have the app open. Though there's a very dilated colon here. And it narrows down for the distal, maybe 6 centimeters, um, and it stays narrow in all images. Um, the other thing I noticed, and this is something very, very important to look for, is there seems to be space between the hollow of the sacrum and the pull-through. The presacral space is a little wider than is normal. So, those are my observations, and now I'd like to know maybe um Jason or Rebecca's opinion of what those observations mean. No, I agree with your description. I think it's important, and, and whoever did this study did a great job of getting a lateral of that rectum, cause it really gives you a lot of information. Um, like Mark said, I think we're trained to look at that space between the sacrum. And the rectum, and then, this looks to be increased uh uniformly throughout this narrowed portion of the most distal part of what I'd call the neorectum for this patient. But Rebecca, why don't you tell us exactly what, uh, what happened there? Yeah. So, um, the, uh, this contrast study, interestingly, because the child required so much sedation to do irrigations, this was actually a contrast study that was performed in the operating room prior to another irrigation, um, with the examination under anesthesia. And so, um, You can see in this study also that um there's a good seal, so all the contrast is not leaking out. The catheter also has not been inserted too high or the balloon is, it's not put under too much pressure because some of the contrast studies if there's um uh basically the low pathology cannot be ascertained easily on a contrast study. And so I think they did a good job on this study. Um, radiology joined us and we're able to get a lot of information here, um, that we could then kind of confirm on exam. So Rebecca, I, I agree with you 100%. What was your working diagnosis theory going into the OR with these images that you already had available to you now? Um, that there was either, uh, so that there's an anatomic issue and it's or a pathologic issue, um, in the lower kind of 6 centimeters of the bowel. Of the pull through. Right, I mean, to me, it looks one of two things. Either you have retained a ganglionosis, you have a pathology problem, or, and again, we don't know if this was a suave, and I'm, with these images, I think it was a suave, seeing that there's so much space between the sacrum and rectum like Mark had mentioned earlier. Is this a pretty long suave cuff that's constricting that most distal portion of neorrectum. Yeah. We pulled through Bal. All right, and unfortunately, we are out of time for this episode. In summary, we discussed a 7-year-old who presented to the emergency room with non-specific findings of Hirschprung's enterocolitis with a history of a previous pull through at 10 days of life at an outside institution. He came to our facility and we did general management with, uh, first starting with rectal irrigation and antibiotics and IV resuscitation. With the most important of these steps really being the rectal irrigation, they are key to the child getting better. And remember you can see uh Hirschsprung's associated enterocolitis protocol that is attached to this podcast within the Stay Current app, so click on the link. And lastly, the contrast enema findings demonstrated a dilated colon with a narrowing at the distal 6 centimeters with a presacral space between the pull through and the sacrum. Remembering the importance on these images to have not only the anterior posterior, but also lateral. With these images, we thought that there was most likely an anatomical or pathological issue with the pull-through in the distal 6 centimeters and that this could be either retained ganglionosis or that this is a swabve that is constricting the most distal portion of the pull-through bowel. All right, and now to our colorectal joke of the day. Did you know that Google Has a platform now for recording your bowel movements. You know its name. Wow, are you, are you serious? There's an app or, or you can do it on, on Google. It's called Google Sheets. That's a good one. All right, that wraps up our colorectal quiz. This was episode 19, Hirschsprung's Disease and the Obstructed Patient, Part 1. next week we'll dive deeper into the rectal exam under anesthesia. Remember, knowledge should be free. This is Amanda Jensen from Riley Children's.

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