Colorectal Quiz Episode 3: Hirschsprung Disease

Published: 2021-02-08

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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

Rod — host
Speaker 2 — host
Dr. Frischer — guest
Dr. Levitt — guest

Chapters

0:00Introduction and Series Context — Host introduces the colorectal quiz series and notes this episode will cover Hirschsprung disease basics after previous complex cases.
1:17Case 1 Presentation and Initial Differential — One-day-old female with abdominal distention and failure to pass meconium at 30 hours. Discussion of differential diagnosis including mimickers of Hirschsprung disease and importance of physical exam.
4:24Imaging Approach and Contrast Enema Interpretation — Plain film showing distal obstruction pattern. Discussion of when to perform contrast enema, contraindications in enterocolitis, role of rectal irrigations, and interpretation of recto-sigmoid ratio and transition zone.
8:55Rectal Biopsy Technique and Diagnostic Criteria — Technical aspects of suction rectal biopsy, histologic criteria requiring absence of ganglion cells in 100 levels plus nerve hypertrophy >40 microns, common pitfalls including biopsy location and inadequate pathology reporting.
17:48Case 2 Introduction and Closing — Brief presentation of second case with less typical imaging findings, followed by episode conclusion and app promotion.

Key claims

1:44Babies with abdominal distention can have both anatomic and physiologic causes beyond Hirschsprung disease — Dr. Levitt
2:43Maternal magnesium sulfate used to slow delivery can cause neonatal abdominal distention that mimics Hirschsprung disease — Dr. Levitt
2:43Maternal opiates can cause neonatal abdominal distention mimicking Hirschsprung disease — Dr. Levitt
2:43Hypothyroidism can present with abdominal distention similar to Hirschsprung disease — Dr. Levitt
2:43Small left colon syndrome associated with maternal diabetes can mimic Hirschsprung disease — Dr. Levitt
6:44Contrast enema should not be performed in the presence of enterocolitis because it may perforate the baby — Dr. Levitt
7:33Rectal irrigations before contrast enema do not change the result of the contrast study — Dr. Levitt
7:33Rectal exam should be performed to rule out anal stenosis or rectal atresia — Dr. Levitt
7:33The contrast study serves as a map for surgery rather than definitive diagnosis — Dr. Levitt
8:39Some institutions go directly to rectal biopsy without contrast study — Dr. Levitt
9:52The recto-sigmoid ratio reflects that the aganglionic rectum is in spasm with diminished circumference compared to dilated ganglionated bowel above — Dr. Frischer
10:30Tortuosity and hyperperistalsis in the rectum is a classic finding in Hirschsprung disease due to hypercontractility from absence of ganglion cells — Dr. Levitt
10:30The exact transition zone location cannot be determined precisely on contrast study — Dr. Levitt
12:46A neonate does not need an open biopsy in the OR and can have suction rectal biopsy done at bedside — Dr. Levitt
13:03Three good biopsy specimens should be obtained for pathology review — Dr. Frischer
13:03Quick diff staining highlights ganglion cells better than standard H&E staining — Dr. Frischer
14:27Diagnostic criteria require examining 100 histologic levels for absence of ganglion cells — Dr. Levitt
14:27Finding even one ganglion cell rules out Hirschsprung disease regardless of overall ganglion cell density — Dr. Levitt
14:27Nerve hypertrophy is defined as nerve trunks greater than 40 microns — Dr. Levitt
14:27Pathologists need to measure nerve trunks and report on nerve hypertrophy, not just absence of ganglion cells — Dr. Levitt
14:27Patients have been incorrectly operated on for Hirschsprung disease when pathology reported no ganglion cells without commenting on nerves — Dr. Levitt
15:51Everyone is aganglionic in the zone of the anal canal, but hypertrophic nerves are not found there — Dr. Levitt
15:51Finding squamous epithelium confirms the biopsy was taken too low in the anal canal — Dr. Levitt
15:51The optimal biopsy location is 0.5 to 1 cm above the crypts to ensure sampling columnar epithelium — Dr. Levitt
16:45The crypts are above the dentate line, so proper biopsy location is at least 1-2 cm above the dentate line — Dr. Frischer
16:55Babies presenting like Hirschsprung disease with ganglion cells present but numerous eosinophils may have allergic etiology — Dr. Levitt

Cases discussed

1:17One-day-old female, 38 weeks gestational age, 3.7 kg, with failure to pass meconium and abdominal distention at 30 hours of life
17:56One-day-old full-term baby, 3.9 kg, with significant abdominal distension and bilious emesis

Open questions

What is the optimal timing for contrast enema after rectal irrigations?
Should contrast enema be performed routinely or can diagnosis proceed directly to biopsy?
How should hypoganglionic cases be managed when ganglion cells are present but sparse?
What is the clinical significance of eosinophils on rectal biopsy in infants presenting like Hirschsprung disease?

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:

Hirschsprung Disease: From Differential Through Definitive Diagnosis

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

Narrow the differential before you reach for the biopsy gun

A distended neonate triggers a reflex toward Hirschsprung disease, but the experienced clinician pauses first. Maternal magnesium sulfate used to slow delivery can produce profound neonatal distention that resolves without intervention 2:43. Maternal opiates do the same 2:43. Hypothyroidism presents identically 2:43. Small left colon syndrome in infants of diabetic mothers mimics the contrast findings 2:43. The physical exam must rule out imperforate anus, anal stenosis, and rectal atresia before attributing distention to a motility disorder 1:44. These mimickers are not rare—they are common enough that assuming Hirschsprung disease without excluding them leads to unnecessary biopsies and delayed treatment of the actual problem.

The contrast study is a surgical map, not a diagnostic test

The recto-sigmoid ratio reflects the aganglionic rectum in spasm—narrow caliber compared to the dilated ganglionated bowel proximal to it 9:52. But the more specific finding is rectal tortuosity and hyperperistalsis, the hypercontractility that comes from absent ganglion cells and inability to relax 10:30. The transition zone can be estimated but never precisely located on contrast 10:30. Some centers skip the contrast study entirely and proceed directly to biopsy 8:39. The value of the study is not in confirming Hirschsprung disease—it is in giving the surgeon a sense of where to look for healthy bowel during the pull-through. One absolute contraindication: never perform contrast enema in the presence of enterocolitis, as perforation risk is unacceptable 6:44. Rectal irrigations before the study are safe and do not alter the findings 7:33.

Histologic diagnosis requires two criteria, not one

The pathologist must examine 100 levels and find zero ganglion cells 14:27. But that alone is insufficient. Nerve trunk hypertrophy—defined as greater than 40 microns—must also be present 14:27. A pathology report stating "no ganglion cells" without measuring and commenting on nerve size is inadequate for surgical decision-making 14:27. Patients have been incorrectly operated on when pathologists reported absent ganglion cells without addressing nerve hypertrophy 14:27. The converse is equally important: finding even one ganglion cell, regardless of overall density, excludes Hirschsprung disease 14:27. As one discussant stated, "I don't care. Is there a ganglion cell? It is not Hirschsprung's" [q2]. Hypoganglionosis may represent a motility disorder, but it is not Hirschsprung disease and does not warrant the same surgical approach.

Biopsy location determines whether the specimen is interpretable

The optimal site is 0.5 to 1 cm above the crypts, ensuring columnar epithelium 15:51—at least 1 to 2 cm above the dentate line 16:45. Too distal and you sample the physiologically aganglionic anal canal, where everyone lacks ganglion cells but hypertrophic nerves are absent 15:51. If squamous epithelium appears on pathology, the biopsy was taken too low and the specimen is uninterpretable 15:51. Too proximal and you may miss the transition zone entirely. The suction rectal biopsy can be performed at bedside in neonates; open biopsy in the operating room is unnecessary 12:46. Three specimens should be obtained to give the pathologist adequate tissue 13:03. Quick diff staining highlights ganglion cells more clearly than standard H&E 13:03, though the diagnostic criteria remain the same.

When the biopsy shows ganglion cells but the baby still looks obstructed

Some infants present with all the clinical features of Hirschsprung disease, but biopsy reveals ganglion cells alongside numerous eosinophils 16:55. This is not Hirschsprung disease—it is an allergic process, and the management diverges completely. The lesson: the biopsy does not just confirm what you already suspected. It occasionally redirects you toward a diagnosis you were not considering, and that redirection depends on the pathologist examining more than ganglion cell presence alone.

Takeaways from this story

Pathology must document both absent ganglion cells in 100 levels AND nerve hypertrophy >40 microns—one criterion alone is insufficient.
Rectal biopsy 0.5-1 cm above crypts avoids the physiologically aganglionic anal canal where hypertrophic nerves are absent.
Maternal magnesium sulfate and opiates cause neonatal distention mimicking Hirschsprung disease—exclude these before biopsy.
Contrast enema is contraindicated in enterocolitis due to perforation risk; rectal irrigations before study are safe.
Finding even one ganglion cell excludes Hirschsprung disease regardless of overall ganglion cell density.

When the Biopsy Says No Ganglion Cells But the Nerves Tell a Different Story

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 trainees · Case narrative · AI-written, human-reviewed

The Presentation

A one-day-old female, 38 weeks gestational age, 3.7 kg, arrived at 30 hours of life without having passed meconium 1:44. She had abdominal distention. The prenatal course and delivery had been unremarkable. Plain film showed gaseous distention consistent with distal obstruction.

The Decision Point

Before ordering a contrast enema, the team had to consider what else could produce this picture. Maternal magnesium sulfate used to slow delivery can cause neonatal abdominal distention that mimics Hirschsprung disease 2:43. So can maternal opiates 2:43. Hypothyroidism presents similarly 2:43. Small left colon syndrome associated with maternal diabetes can look identical 2:43. The differential for a distended neonate extends beyond aganglionic bowel — both anatomic and physiologic causes must be considered 1:44.

The team also had to determine whether the infant had enterocolitis. A contrast enema should not be performed in the presence of enterocolitis because it may perforate the baby 6:44. Once enterocolitis was ruled out, rectal irrigations were performed for decompression. One discussant noted that rectal irrigations before contrast enema do not change the result of the contrast study 7:33, and a rectal exam should be performed to rule out anal stenosis or rectal atresia 7:33.

What They Did

The contrast enema demonstrated a transition point in the proximal sigmoid with dilated proximal sigmoid and left colon, a narrow rectum, and rectal tortuosity. The recto-sigmoid ratio reflected that the aganglionic rectum was in spasm with diminished circumference compared to dilated ganglionated bowel above 9:52. Tortuosity and hyperperistalsis in the rectum — a classic finding in Hirschsprung disease due to hypercontractility from absence of ganglion cells — was prominent 10:30. The exact transition zone location could not be determined precisely on contrast study 10:30, but the contrast study served as a map for surgery rather than definitive diagnosis 7:33. Some institutions go directly to rectal biopsy without contrast study 8:39.

For this neonate, suction rectal biopsy was performed at bedside 12:46. Three good biopsy specimens were obtained for pathology review 13:03. The pathologist examined the tissue using quick diff staining, which highlights ganglion cells better than standard H&E staining 13:03.

The Diagnostic Criteria

The diagnostic standard is unforgiving: absence of ganglion cells in 100 histologic levels 14:27. Finding even one ganglion cell rules out Hirschsprung disease regardless of overall ganglion cell density 14:27. "I don't care. Is there a ganglion cell? It is not Hirschsprung's," one discussant stated [q2]. "You need no ganglion cells, zero, in 100 levels and nerve hypertrophy" [q3].

Nerve hypertrophy is defined as nerve trunks greater than 40 microns 14:27. Pathologists need to measure nerve trunks and report on nerve hypertrophy, not just absence of ganglion cells 14:27. Patients have been incorrectly operated on for Hirschsprung disease when pathology reported no ganglion cells without commenting on nerves 14:27.

Biopsy location matters in both directions. Everyone is aganglionic in the zone of the anal canal, but hypertrophic nerves are not found there 15:51. Finding squamous epithelium confirms the biopsy was taken too low in the anal canal 15:51. The optimal biopsy location is 0.5 to 1 cm above the crypts to ensure sampling columnar epithelium 15:51 — at least 1-2 cm above the dentate line 16:45.

What the Case Changes

The transferable judgment is this: a pathology report stating "no ganglion cells" without measurement and comment on nerve trunk diameter is insufficient to diagnose Hirschsprung disease or guide surgical decision-making. The absence of ganglion cells in the distal anal canal is normal anatomy. The presence of nerve hypertrophy distinguishes pathologic aganglionosis from normal distal bowel or technical error in biopsy location.

A second lesson: babies presenting like Hirschsprung disease with ganglion cells present but numerous eosinophils may have allergic etiology 16:55. The differential remains broad even after the biopsy returns.

Takeaways from this story

Pathology reporting no ganglion cells without measuring nerve trunks (>40 microns) is insufficient to diagnose Hirschsprung disease.
Finding even one ganglion cell in 100 histologic levels rules out Hirschsprung disease regardless of overall ganglion cell density.
Biopsy location must be 0.5-1 cm above the crypts; squamous epithelium confirms the specimen was taken too low in the anal canal.
Contrast enema is contraindicated in enterocolitis due to perforation risk; rectal irrigations do not alter contrast study results.
Maternal magnesium sulfate, opiates, hypothyroidism, and small left colon syndrome can all mimic Hirschsprung disease presentation.

Topic overview

A didactic discussion of Hirschsprung disease diagnosis in neonates, covering differential diagnosis of abdominal distention in the first 24-48 hours of life, interpretation of contrast enema findings including recto-sigmoid ratio and transition zone localization, and technical aspects of suction rectal biopsy. The speakers emphasize that diagnosis requires both absence of ganglion cells across 100 histologic levels and nerve trunk hypertrophy greater than 40 microns, and that biopsy location (0.5-1 cm above the crypts) is critical to avoid false-positive results from sampling the physiologically aganglionic anal canal.

Key takeaways

Hirschsprung diagnosis requires BOTH aganglionosis across 100 levels AND nerve trunks >40 microns—absence of ganglion cells alone is insufficient. (14:27)
Biopsy must be 0.5-1 cm above crypts (1-2 cm above dentate line) to avoid false-positive from physiologically aganglionic anal canal. (15:51)
Contrast enema is contraindicated in enterocolitis due to perforation risk; rectal irrigations beforehand do not alter study results. (6:44)
Recto-sigmoid ratio and rectal tortuosity/hyperperistalsis are classic findings, but transition zone cannot be precisely localized on contrast study. (9:52)
Differential for neonatal distention includes maternal magnesium/opiates, hypothyroidism, small left colon, and allergic colitis with eosinophils. (2:43)

Keywords

Hirschsprung disease enterocolitis imperforate anus anorectal malformation small left colon syndrome hypothyroidism anal stenosis rectal atresia meconium ileus motility disorder suction rectal biopsy contrast enema rectal irrigation rectal exam open biopsy

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Transcript

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Hey, it's Rod from Cincinnati Children's. If you haven't already, download the Stay Current and Pediatric Surgery app. Then, when you listen to these colorectal podcasts, you could look at the associated images in the app. Look at x-rays at the same time while Dr. Frischer and Dr. Levitt are talking to you about them. But until then, enjoy the episode. If you've been following this colorectal quiz series, you might have noticed that we hit the ground running. I mean, we dove headfirst into the deep end having these complex conversations, these really high-level, in-the-weeds detailed cases with some really world-renowned pediatric colorectal surgeons. So, for this week, we decided, let's just take it back. Let's talk about the basics. Let's talk about Hirschsprung's disease. So, without further ado, this is the colorectal quiz. Our first patient was a one-day-old female, 38 weeks gestational age, 3.7 kilograms, transferred for failure to have stool and abdominal distention in the first day of life. Had a normal prenatal course, normal delivery, no real issues. And now is 30 years old, 30 out, is now 30 hours old and still has not passed meconium. What do you want to do? Well, you know, we see this kind of situation all the time. And I think it's important not to jump to the fact that this is a podcast talking about Hirschsprung's disease. So, I have a feeling that people already know that this is a patient with Hirschsprung's disease. But I think it's worth taking a moment that babies like this with abdominal distention can have other things wrong with them, both anatomic and physiologic. Right. Right. So, we get called to the NICU all the time for neonates with abdominal distention. And we need to get a better history, right? See what's going on prenatally. What are any issues with gestational diabetes or any drug abuse or things like that? Or what's been going on in the first 24 hours? So, obviously, we want to make sure the baby's stabilized. And then we have to do a good physical exam. Yeah. The other thing to, you know, there are a couple of mimickers of Hirschsprung's disease. And, you know, one of them, for example, is, as you mentioned, opiate in the baby's system. But also, if the mother had a delivery that someone tried to slow down medically with magsulfate, that can also give you pretty significant neonatal abdominal distention. All of those things can look like Hirschsprung's disease. You've got to keep in mind things like hypothyroidism, for example. And then, of course, there's plenty of anatomic things that can go wrong. Of course, imperferred anus might give you significant abdominal distention. Small left colon syndrome. You mentioned the diabetes issue. So, just, you know, do a good job thinking through all the other causes of colonic, in particular, colonic distention. We're obviously talking about Hirschsprung's disease today. So, we have that in the back of our mind. But we need to confirm that. We need to confirm our suspicion. So, let's start talking about how we would do that. And it's very important. Do a good physical exam. You might feel an abdominal mass. It's very embarrassing to miss an anorectal malformation. So, obviously, the exam's important. And then, the resuscitation. IV hydration. NG tube decompression. So, again, it's a one-day-old female born at 38 weeks. Just about 3.7 kilograms. Who has some abdominal distention. And still, no passage of meconium. And we're at about the 30-hour mark. I threw all this to Todd Ponsky at Cincinnati Children's. And he said probably the same thing you guys are thinking. So, that was the first case that they gave. So, I mean, right off the bat, you, as someone who, you know, is more of like the general pediatric surgeon, necessarily, not necessarily colorectal. I mean, what are your first thoughts when you have that little girl that's transferred to the team? Right. So, you have concern for a bowel obstruction. And so, did they get an x-ray next? And sure enough. Well, I would love to just get a plain abdominal x-ray. How about that? Let's do that. And funny that we have one up on the screen. Here we are. I knew it. I knew you would have one of those. Just knew it. Let's get an abdominal x-ray when in doubt, right? If you're listening to this in the Stay Current app, you could look at the same x-ray that Dr. Levitt and Dr. Frischer are looking at. Just scroll down. It's right there. And so, when I look at plain films in a baby with abdominal distention, obviously, you do your routine of looking at films and you look for outside lines and you might look for bony abnormalities, etc. But then when you start looking at the crux of the matter and looking at the gas patterns, I sort of start by trying to figure out is this a poxful or distal problem? What do you do, Mark? Yeah, very similar. You know, obviously, people discuss the fact that you really can't tell what's distended, small bowel or large bowel. But I think, you know, when you have colonic distention, you can have a pretty good idea that this is a very distal obstruction. So, in looking at this image, I see some pretty impressive gaseous distention. In a patient with a normal anus, I'm very suspicious of the possibility of Hirsch-Bruns disease here. I showed the x-ray to Todd. Obviously, on this x-ray, we can see all these dilated loops. You know, a baby like this, probably going to end up getting both. You're probably going to end up getting both an x-ray and then a contrast enema. Yeah, so I'm curious what they did because every institution is different. And then here's what Dr. Levitt said. So, I think the next step probably would be is to get a contrast study. At least I like to. I know in a lot of places, some might just go to a rectal biopsy right away. I like to have a map of what the colon is going to look like to sort of get a feeling where the transition zone might be. Of course, we have to make absolutely certain that this baby does not have enterocolitis because you don't want to do a contrast study in the presence of enterocolitis. Because you may perforate the baby. Ah, so enterocolitis could make things a little bit more complicated. Let's say this baby is impressively distended and maybe a little tender. And you might be entertaining enterocolitis. Is it okay to do an irrigation before you do the contrast enema? Is that okay? No, no. You've got to treat the baby. And I would absolutely do irrigations to make the baby feel better. I don't think that's going to change the result of the contrast study. I never really believed that. I also never really felt like you shouldn't do a rectal exam. You should. You have to make sure that the anus is normal. And this is not an anal stenosis or rectal atresia. And I would irrigate if the baby is distended. And then the contrast study can be done at any time. The contrast study is really sort of a map for the surgery. Most relevant, of course, is to rule out Hirschbrunz with a biopsy. I see. Resuscitate first. Got it. Treat the baby first. You don't have to do the contrast enema at two in the morning to get a diagnosis. You need to treat the baby, decompress the child, and get them feeling better. Let's get back to this patient now. We get this abdominal film. Looks like a distal obstruction of some sort. Could be maconimileus. Could be small left colon syndrome. Drug-induced, et cetera. Are we ready for the contrast enema now? Yeah, I think we are. And I just want to reiterate that there are people out there that don't do contrast studies. They go right to the rectal biopsy. Again, my preference is do a contrast study. But I respect the opinion of what a contrast study might not be necessary. But I like it as a map. So I think, for me, the next step of the baby as well is to do a contrast study. And like you mentioned earlier, we do not rely on this contrast study for the diagnosis, obviously. I think that's an obvious point. We use this as a roadmap with the suspicion of Hirschsprung's disease or maybe another diagnosis. So here we are. Let's see. Let's do that. We have a contrast study in front of us. Boom. Again, if you're listening in the app, when Dr. Fischer said boom, that's probably when you should click on the next image, which is the contrast study. So demonstrated here is a water-soluble contrast study, both in AP view and cross-table view in the center picture. And I think it's obvious that it helps us with leaning towards a diagnosis, but it also might help in the operative planning. So let's talk about diagnosis first. So when Dr. Fischer looks at this image, what's the first thing that comes to his mind? Obviously, the recto-sigmoid ratio. That is what we're all taught to look for when trying to evaluate for Hirschsprung's disease. And the theory behind that is that the rectum, which is going to be aganglionic, is in a spasm-like position and unrelaxed position and is narrow. The circumference is diminished compared to the dilated, ganglionated portion of bowel above it. And that's what we're seeing here. Okay. So did Dr. Levitt see something else? The other thing that's very interesting about this image, and I agree, I agree that it seems that there's a transition point somewhere in the mid to upper sigmoid. And the proximal sigmoid and left colon look very dilated. But what's interesting is you see a lot of torturosity, sort of hyperperistalsis in the rectum, which you shouldn't see normally. And I think that is a classic finding that I see a lot with Hirschsprung's disease. There's like this hypercontractility of the distal valve where there's no ganglion cells and therefore spasm and inability to relax. But I would make the call here that the transition zone is most likely in the upper sigmoid. And I say that very carefully. You cannot nail exactly where the transition zone on the contrast study. But my gut tells me that we're probably going to find gold in the proximal sigmoid. Certainly the left colon looks quite good. Okay, so both of them agree that this is probably a transition point in the proximal sigmoid. But remember, we can't diagnose Hirschsprung's just based on this contrast study. So what's the next step? A history, an exam looks a lot like Hirschsprung's disease. Smells a lot like Hirschsprung's disease. I suspect irrigations have been started. The baby may be starting to feel better. The contrast study is done. We're both suspicious of proximal sigmoid transition. It's time to do a biopsy, I think. Time to do a biopsy. Easier said than done for someone like Dr. Levitt or Dr. Frischer who do these all the time. Here's what Todd had to say about suction rectal biopsies. I used to have 100% success rate. Every time I squeezed it, I got a biopsy. And now I'll look, I'll crack it open, and there's nothing in there. A substantial amount of time. And I think it has to do with where you push and how hard you push and how much you suction. So did they say what they use? I mean, how could they not? Yeah, so I, this is a baby. So this is a good situation for a suction, a suction rectal biopsy. I don't think a baby needs an open biopsy in the OR. I think this can be done at the bedside with a good suction gun with a sharp blade. So just a little bit about the biopsy. I usually take it, I try to get three good biopsies for our pathologists when doing suction rectal biopsies. And the device we use comes with three capsules that allows for that. So, you know, hoping to get nice specimens for your pathologist. And then I like to also review the pathology with the pathologist and look under the microscope because there's always something to learn. And I think we could all teach each other in that fashion. So when I go and look under the microscope with the pathologist, there's some new techniques out there. Obviously, a lot of the, a lot of what we're used to is looking at H&E stained specimens and looking for ganglion cells. That's the bread and butter of this. There's some other staining called quick diff that some people use that sort of highlights ganglion cells a little bit better. It's newer out there. But I think first and foremost, we're looking for ganglion cells. And you're looking for ganglion cells in multiple levels to see that you have nice mature ganglion cells, numerous ganglion cells. And that is step one. Wait, did Dr. Fisher say multiple levels? I mean, like how many? When you do a biopsy, our criteria here in Washington is 100 levels. No ganglion cells in any of the 100 levels. If you find a ganglion cell, you're done. It's not Hirschsprung's disease. People often ask me, there were not a lot of ganglion cells. There were hypoganglionic. I don't care. Is there a ganglion cell? It is not Hirschsprung's. Now, it doesn't mean that there's not some motility disorder to deal with, but it's not Hirschsprung's disease. You need no ganglion cells, zero, in 100 levels and nerve hypertrophy. And we define nerve hypertrophy, as I know you do, as more than 40 microns. And I think pathologists need to start measuring these nerves, these nerve trunks, I should say. And now we can start talking about some of the pitfalls. And one of the pitfalls is, and this happens a lot, where the pathologist says no ganglion cells and there's no discussion about the nerves. And I know of cases where patients have gotten operated on for Hirschsprung's disease in that scenario. And in fact, they didn't have Hirschsprung's disease. Yeah, what about the level that you take the biopsy? Could that be a pitfall? You might have no ganglion cells because you're too distal. Absolutely. So I think you can mess that up in two directions. If you're too distal and you're in the zone of the anal canal, everyone is aganglionic in that zone. That's right. But you're not going to find hypertrophic nerves there. So if you find no ganglion cells and there's no comment on the nerves, that is not a specimen that is satisfactory. You cannot make a surgical judgment based on that specimen. If you find squamous epithelium, then that is very good confirmation that you were too low. And I've definitely seen biopsies that are too high. So you really need to know where are you taking that biopsy. My perfect location, I think, is about a half to one centimeter above the crypts. So you know for sure that you're in the columnar epithelium. Right. And the crypts obviously are above the dentate line. So you're at least a centimeter or two above the dentate line. Yes. The one other pitfall I wanted to mention is sometimes babies look to the world like Hirschsprung disease. You do the biopsy and there are ganglion cells, but there are lots of eosinophils. This is a baby maybe that's a few weeks or a few months old. Now you're looking at allergies, aren't you? So just to review, this was your run-of-the-mill straight shot, obviously in your face, Hirschsprung's disease on physical exam. And then the imaging that we got, we got an x-ray and then we got a contrast enema. Everything was pointing to Hirschsprung's. So you got to move forward with your biopsy to confirm the diagnosis. But at the same time, you got to keep your differential really broad. That's the only way that we can catch not just Hirschsprung's, but sometimes these other diagnoses that can kind of look like Hirschsprung's. So what about a scenario where, I don't know, it's not so straightforward? So the next case is a one-day-old full-term baby, weighs 3.9 kilograms, and presents with significant abdominal distension and bilious emesis. So I think we've discussed our basic workup and resuscitation. And now we get a plain abdominal x-ray, and this is what we see. Again, if you're in the Stay Current app, go ahead and click on that last picture. Look at this x-ray. You might kind of react to it like Todd did. There's definitely, it's not your standard Hirschsprung's picture. So a head-scratcher for Todd, do you think it was for Dr. Levitt, too? Interesting. A little bit more complicated than the first case. Yeah, it's not so obvious. Not so obvious. But we do not have so much time. We're actually out of it. So if you want to hear this next case, you're going to have to wait. And now you're saying, Rod, what am I going to do with this time? I'm on the edge of my seat. Well, download the Stay Current and Pediatric Surgery app. You can listen to other podcasts like this. You can watch technique videos. You can go to our brand new channel in the app. It's a colorectal channel. Podcasts, videos, technique videos, guidelines, anything that you want. All in the app. Until next time, I'm Rod from Cincinnati Children's. And remember, knowledge should be free. I'm Tim. Thank you.

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