The Colorectal Quiz Episode 10: Total Colonic Hirschsprung Disease Part 1

Published: 2021-04-12

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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 Gerardo — host
Amanda Jensen — host
Jason Fisher — guest
Mark Levitt — guest

Chapters

0:00Introduction and Case Presentation — Hosts introduce the topic of total colonic Hirschsprung disease and present a 10-month-old with delayed diagnosis, severe constipation, and history of meconium plug at birth.
1:43Differential Diagnosis and Missed Opportunities — Discussion of why the diagnosis was missed, differential for failure to pass meconium, and the recommendation that 10% of meconium plug cases warrant biopsy for Hirschsprung disease.
5:05Imaging Review — Review of contrast enema at birth and at 10 months showing cylindrical, amorphous colon without classic rectosigmoid narrowing, distinguishing total colonic from typical Hirschsprung presentation.
7:13Biopsy Strategy and Operative Management — Discussion of suction versus full-thickness biopsy timing, intraoperative colonic mapping when no transition zone is visible, and decision to place diverting ileostomy after frozen section confirmed aganglionosis.
10:27Summary and Conclusion — Case summary and preview of next episode covering definitive management of total colonic Hirschsprung disease.

Key claims

3:1310% of patients with meconium plug have Hirschsprung disease — Mark Levitt
4:07Failure to pass meconium in newborns is most commonly caused by Hirschsprung disease, meconium plug syndrome, meconium ileus, or anorectal malformation — Rod Gerardo
4:21Rare causes of failure to pass meconium include small left colon syndrome, hypothyroidism, opiates in the newborn system, magnesium sulfate exposure, milk protein allergy, and microcolon intestinal hypoperistalsis syndrome — Amanda Jensen
6:33In total colonic Hirschsprung disease, contrast enema shows cylindrical, amorphous colon without the classic rectosigmoid narrowing seen in typical Hirschsprung disease — Rod Gerardo
7:26Age cutoff for switching from suction rectal biopsy to full-thickness biopsy is around 6 months — Jason Fisher
7:46Suction biopsies are problematic when they do not provide a definitive diagnosis, requiring subsequent formal biopsy in the operating room — Mark Levitt
8:03Rectal biopsies should be attempted pre-operatively before proceeding to the operating room — Amanda Jensen
8:38If a patient is doing well and can be managed with irrigations, final diagnosis of Hirschsprung disease should be obtained through rectal biopsy before entering the abdomen — Jason Fisher
8:53If a patient is not doing well with irrigations, a procedure to relieve pressure such as ileostomy or leveling colostomy is necessary — Amanda Jensen
9:04Loop ostomies should be avoided in Hirschsprung disease because stool may spill into the non-functional part of the colon — Amanda Jensen
9:13Standard intraoperative biopsy sites include rectosigmoid, proximal sigmoid, left colon, splenic flexure area, transverse colon, right colon, and hepatic flexure area — Jason Fisher
9:35When no transition zone is visible during surgery, additional biopsies should be obtained at multiple sites along the colon — Amanda Jensen
10:09A definitive pull-through procedure should not be performed based solely on frozen section if there is uncertainty about the diagnosis — Amanda Jensen
3:30In meconium plug cases, the initial contrast enema may show what appears to be a meconium plug but is actually a segment of Hirschsprung disease — Amanda Jensen
3:53After meconium passes in suspected Hirschsprung cases, obtaining another contrast image can reveal the characteristic appearance of Hirschsprung disease — Amanda Jensen

Cases discussed

1:4310-month-old with delayed diagnosis of total colonic Hirschsprung disease

Open questions

What is the optimal definitive surgical approach for total colonic Hirschsprung disease (to be discussed in part 2)?
Should all neonates with meconium plug undergo routine rectal biopsy given the 10% Hirschsprung association?
What is the optimal timing for definitive pull-through after diverting ostomy in total colonic cases?

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:

Total Colonic Hirschsprung Disease: Recognition, Diagnosis, and Staged Management

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

Meconium plug demands biopsy

A newborn who passes meconium after contrast enema looks like a solved problem. It is not. 10% of patients with meconium plug have Hirschsprung disease 3:13. The initial contrast study may show what appears to be a plug but is actually an aganglionic segment 3:30. After the meconium passes, a repeat contrast image can reveal the characteristic appearance of Hirschsprung disease 3:53. The teaching point: successful passage of meconium does not close the differential. Biopsy before discharge, or accept that you may spend months managing a distended, poorly feeding infant whose diagnosis you missed 3:13.

Total colonic disease lacks the classic transition zone

In rectosigmoid Hirschsprung disease, contrast enema shows a narrowed rectum transitioning to dilated sigmoid — the image is diagnostic. Total colonic Hirschsprung disease shows a cylindrical, amorphous colon without that narrowing 6:33. The rectum and sigmoid are the same caliber [q3]. Radiologists may read this as normal or meconium plug because the expected transition zone is absent. The colon looks wrong — featureless, uniform — but not in the way trainees are taught to recognize. This is why the diagnosis gets missed at birth and presents months later with obstruction.

Age and clinical stability determine biopsy approach

Around 6 months of age, switch from bedside suction biopsy to full-thickness biopsy in the operating room 7:26. Suction biopsies are useful when they provide a definitive answer; when they do not, you have added a procedure without advancing the diagnosis 7:46. A 10-month-old with this history needs a definitive answer, which means full-thickness tissue. The principle: attempt rectal biopsy pre-operatively before entering the abdomen 8:03. If the patient is stable and can be managed with irrigations, obtain that final diagnosis through biopsy before committing to laparotomy 8:38. If irrigations are failing and the patient is decompensating, the clinical situation forces your hand — proceed to the operating room for pressure relief 8:53.

Avoid loop ostomies in Hirschsprung disease

When diversion is necessary, construct an end ostomy, not a loop. Loop ostomies allow stool to spill into the non-functional distal colon 9:04, defeating the purpose of diversion. If you are creating an ostomy in suspected or confirmed Hirschsprung disease, commit to complete diversion.

Map the colon systematically when no transition is visible

Standard intraoperative biopsy sites include rectosigmoid, proximal sigmoid, left colon, splenic flexure area, transverse colon, right colon, and hepatic flexure area 9:13. When you cannot identify a transition zone visually — and in total colonic disease you will not — obtain biopsies at multiple levels to map the extent of aganglionosis 9:35. Frozen section that shows no ganglion cells anywhere is not a mandate to proceed with pull-through. If there is uncertainty about the diagnosis or the extent of disease, place a diverting ostomy and wait for permanent sections 10:09. You can return for definitive repair once pathology clarifies what you are dealing with. The error is not deferring the pull-through; the error is performing a definitive procedure based on incomplete information.

The principle the discussants most emphasized

Do not perform a definitive pull-through based solely on frozen section if there is uncertainty about the diagnosis 10:09. Frozen section confirms aganglionosis, but it does not always clarify extent, and it can be equivocal in difficult cases. Diversion is reversible. An inadequate pull-through is a problem you will be managing for years. When the intraoperative picture does not match your pre-operative plan, the correct answer is to stop, divert, and return with better information.

Takeaways from this story

10% of neonates with meconium plug have Hirschsprung disease; biopsy before discharge to avoid missing the diagnosis.
Total colonic Hirschsprung lacks the classic rectosigmoid narrowing and may be misread as normal on contrast enema.
Around 6 months, switch from suction to full-thickness biopsy for definitive diagnosis in uncertain cases.
Avoid loop ostomies in Hirschsprung disease; stool spillage into non-functional colon defeats the purpose of diversion.
Never perform definitive pull-through based solely on frozen section if extent of disease is uncertain; divert and wait for permanent pathology.

Total Colonic Hirschsprung Missed at Birth, Diagnosed at Ten Months

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

The Presentation

A ten-month-old presented to the emergency department with severe abdominal distension and intractable constipation requiring MiraLax cleanouts that had failed to resolve the problem [c1:case1]. The history was revealing: the infant had failed to pass meconium at birth, attributed at the time to meconium plug syndrome, and no suction rectal biopsy had been performed in the neonatal period [c1:case1]. The initial contrast enema at birth had shown an amorphous, cylindrical colon with air in the small bowel — an image that one of the discussants acknowledged could easily have been read as normal or simply meconium plug, though in retrospect it "does not look right" [q2]. At ten months, imaging showed dilated bowel and a significant stool burden throughout a cylindrical colon without the classic rectosigmoid narrowing that defines typical Hirschsprung disease 6:33.

The Missed Opportunity

The first decision point had come and gone nine months earlier. When a newborn passes a meconium plug, 10% of those patients have Hirschsprung disease 3:13. One discussant was direct about the stakes: "you do not want to have that kid limp along for the next few months, constipated, not eating well, distended, and in fact you missed the diagnosis of Hirschprung's" [q1]. The mechanism is worth understanding: in meconium plug cases, the initial contrast enema may show what appears to be a meconium plug but is actually a segment of Hirschsprung disease 3:30. After the meconium passes, obtaining another contrast image can reveal the characteristic appearance of Hirschsprung disease 3:53. Neither the biopsy nor the repeat imaging had been done.

The Diagnostic Path at Ten Months

At ten months, the team faced a choice between bedside suction rectal biopsy and full-thickness biopsy in the operating room. The age cutoff for switching from suction to full-thickness biopsy is around six months 7:26, and this patient was well past that threshold. More fundamentally, the discussants agreed that with this history, a definitive diagnosis was essential. As one put it, "suction biopsies are great except when you don't have a definitive diagnosis and then you have to do something more definitive and then you need to go to the OR and do a formal biopsy" [q5]. The principle is clear: rectal biopsies should be attempted pre-operatively before proceeding to the operating room 8:03, but if a patient is doing well and can be managed with irrigations, the final diagnosis of Hirschsprung disease should be obtained through rectal biopsy before entering the abdomen 8:38. This patient was not doing well with irrigations, forcing the team's hand toward a procedure to relieve pressure 8:53.

Intraoperative Findings

The team proceeded with laparoscopic colonic mapping, obtaining biopsies at standard sites: rectosigmoid, proximal sigmoid, left colon, splenic flexure area, transverse colon, right colon, and hepatic flexure area 9:13. When no transition zone is visible during surgery, additional biopsies should be obtained at multiple sites along the colon 9:35 — exactly what was done here. Frozen section showed no ganglion cells throughout the colon [c1:case1]. The team placed a diverting loop ileostomy and stopped, performing no definitive pull-through procedure [c1:case1].

The Judgment Call

That decision — to place an ostomy and wait rather than proceed to pull-through on frozen section alone — was the critical judgment. A definitive pull-through procedure should not be performed based solely on frozen section if there is uncertainty about the diagnosis 10:09. The discussants endorsed this approach without reservation. Permanent pathology would clarify what frozen section suggested, and a second operation could always be scheduled. The choice of loop ileostomy drew comment, however: loop ostomies should be avoided in Hirschsprung disease because stool may spill into the non-functional part of the colon 9:04.

What the Case Changes

The transferable judgment is this: failure to pass meconium attributed to meconium plug demands either rectal biopsy or repeat contrast imaging after the plug passes, because 10% of these infants have Hirschsprung disease 3:13. When Hirschsprung is total colonic, the contrast enema will not show the classic rectosigmoid narrowing — it will show a cylindrical, amorphous colon 6:33, an appearance that can be mistaken for normal. At ten months, with severe symptoms and ambiguous imaging, full-thickness biopsy in the operating room is the correct path 7:26. And when frozen section shows aganglionosis throughout the colon without a visible transition zone, the correct response is a diverting ostomy and patience, not a definitive procedure based on intraoperative pathology alone 10:09. The outcome of this case was not discussed.

Takeaways from this story

10% of newborns with meconium plug have Hirschsprung disease — biopsy or repeat contrast after plug passes is mandatory.
Total colonic Hirschsprung shows cylindrical colon without rectosigmoid narrowing, easily mistaken for normal on contrast enema.
Switch from suction to full-thickness rectal biopsy around 6 months; definitive diagnosis required before entering abdomen if patient stable.
When frozen section shows aganglionosis without visible transition zone, place diverting ostomy and wait for permanent pathology — no pull-through on frozen alone.
Avoid loop ostomies in Hirschsprung — stool spills into non-functional colon; obtain biopsies at multiple standard sites when no transition visible.

Total Colonic Hirschsprung Diagnosed at Ten Months After Neonatal Meconium Plug

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

The Missed Opportunity

A ten-month-old presented to the emergency department with severe abdominal distension and intractable constipation requiring MiraLax cleanouts 3:13. The history was revealing: failure to pass meconium at birth, attributed to meconium plug syndrome, with no suction rectal biopsy performed in the neonatal period. The initial contrast enema had shown an amorphous, cylindrical colon with air in the small bowel [case1]. At ten months, imaging showed dilated bowel and significant stool burden throughout a cylindrical colon without the classic rectosigmoid narrowing seen in typical Hirschsprung disease 6:33.

The neonatal presentation should have prompted further investigation. When a newborn passes a meconium plug, 10% have underlying Hirschsprung disease 3:13. As one discussant noted, "you do not want to have that kid limp along for the next few months, constipated, not eating well, distended, and in fact you missed the diagnosis of Hirschprung's" [q1]. The initial contrast study itself was deceptive — what appeared to be a meconium plug was likely a segment of aganglionic colon 3:30. After meconium passes in such cases, obtaining another contrast image can reveal the characteristic appearance of Hirschsprung disease 3:53.

The Decision Point

At ten months, with severe symptoms and concerning imaging, the team faced a diagnostic choice: bedside suction rectal biopsy versus full-thickness biopsy in the operating room. The age cutoff for switching from suction to full-thickness biopsy is around six months 7:26. One discussant explained his reasoning: "suction biopsies are great except when you don't have a definitive diagnosis and then you have to do something more definitive and then you need to go to the OR and do a formal biopsy" [q5]. With this history and the need for a reliable diagnosis, the team chose the operating room [case1].

But a critical principle applies here: rectal biopsies should be attempted pre-operatively before proceeding to the operating room 8:03. If a patient is doing well and can be managed with irrigations, the final diagnosis of Hirschsprung disease should be obtained through rectal biopsy before entering the abdomen 8:38. This patient's clinical deterioration forced the issue — when irrigations fail, a procedure to relieve pressure such as ileostomy or leveling colostomy becomes necessary 8:53.

Intraoperative Mapping

The team performed laparoscopic colonic mapping with biopsies at standard sites: rectosigmoid, proximal sigmoid, left colon, splenic flexure area, transverse colon, right colon, and hepatic flexure area 9:13. When no transition zone is visible during surgery, additional biopsies should be obtained at multiple sites along the colon 9:35. Frozen sections showed no ganglion cells throughout the colon [case1].

Faced with this finding, the team made a conservative choice: they placed a diverting loop ileostomy without performing a definitive pull-through procedure [case1]. This decision reflects an important principle — a definitive pull-through procedure should not be performed based solely on frozen section if there is uncertainty about the diagnosis 10:09. The team could wait for permanent pathology and return another day for definitive repair.

One technical note: loop ostomies should be avoided in Hirschsprung disease because stool may spill into the non-functional part of the colon 9:04. An end ileostomy would have been preferable.

What the Case Changes

The differential diagnosis for failure to pass meconium in newborns includes Hirschsprung disease, meconium plug syndrome, meconium ileus, and anorectal malformation as the most common causes 4:07. Rare causes include small left colon syndrome, hypothyroidism, opiates in the newborn system, magnesium sulfate exposure, milk protein allergy, and microcolon intestinal hypoperistalsis syndrome 4:21. But the critical judgment is this: when meconium plug is diagnosed, biopsy for Hirschsprung disease before discharge.

Total colonic Hirschsprung does not present with the classic contrast enema findings. One discussant, reviewing the ten-month imaging, acknowledged that the lack of narrowing at the rectum compared to the sigmoid could lead to the study being called normal 6:33. The colon remains cylindrical and amorphous 6:33. Recognizing this variant requires clinical suspicion, not radiographic certainty.

The outcome of this case was not discussed beyond the placement of the ileostomy. The definitive management of total colonic Hirschsprung — whether ileoanal pull-through, ileosigmoid anastomosis with patch, or another approach — remained ahead.

Takeaways from this story

10% of newborns with meconium plug have Hirschsprung disease — biopsy before discharge to avoid delayed diagnosis.
Total colonic Hirschsprung lacks classic rectosigmoid narrowing on contrast enema, appearing cylindrical and amorphous instead.
Attempt pre-operative rectal biopsy when possible; reserve OR biopsy for patients failing conservative management.
Never perform definitive pull-through based solely on frozen section if diagnosis is uncertain — ostomy and return later.
Avoid loop ostomies in Hirschsprung disease; stool spillage into aganglionic colon complicates subsequent management.

Topic overview

A 10-month-old with delayed diagnosis of Hirschsprung disease presented with severe abdominal distension and constipation after failing to pass meconium at birth (attributed to meconium plug without biopsy). Contrast enema showed cylindrical, amorphous colon without classic rectosigmoid narrowing. Intraoperative biopsies found no ganglion cells throughout the colon, confirming total colonic Hirschsprung disease; a diverting ileostomy was placed. The discussion emphasizes that 10% of neonates with meconium plug have Hirschsprung disease and should undergo rectal biopsy, and that total colonic cases present differently on imaging than classic rectosigmoid disease.

Key takeaways

10% of neonates with meconium plug have Hirschsprung disease; rectal biopsy is mandatory to rule it out. (3:13)
Total colonic Hirschsprung shows cylindrical colon on enema, not the classic rectosigmoid narrowing of typical disease. (6:33)
Avoid loop ostomies in Hirschsprung—stool spills into non-functional colon. Use end ileostomy or leveling colostomy. (9:04)
Never perform definitive pull-through on frozen section alone if uncertain; obtain permanent histology first. (10:09)
If no transition zone is visible intraoperatively, biopsy rectosigmoid, sigmoid, left colon, flexures, and right colon. (9:13)

Keywords

Hirschsprung disease total colonic Hirschsprung disease total colonic aganglionosis meconium plug syndrome meconium ileus anorectal malformation small left colon syndrome hypothyroidism milk protein allergy microcolon intestinal hypoperistalsis syndrome constipation abdominal distension suction rectal biopsy full-thickness rectal biopsy contrast enema

Hashtags

#HirschsprungDisease #PediatricSurgery #ColorectalQuiz #MeconiumPlug

Transcript

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Hey there listeners, it's Rod Gerardo and Amanda Jensen from Cincinnati Children's. Have you downloaded the new version of the stay current pediatric surgery app? It's in the Apple App Store, it's in the Google Play Store. The reason I bring it up is that if you're listening to this podcast within the app, you can bring up journal articles, radiological images, or pictures in real time while Doctor Fisher and Doctor Levitt are talking about them. So download the app. We know you're going to love it, but until then, enjoy the episode. In previous episodes, we have talked about Hirschsprung disease. Like so many episodes are about Hirschprung's, but you know what we haven't talked about total colonic a ganglionosis or total clonic Hirschprung's. Amanda, what is that? There is an absence of ganglion cells throughout the entire colon. And as you can imagine, these patients are diagnosed and treated a little bit differently than the Hirschprung patients in our previous episodes. So that's what we're going to talk about. Today, total colonic Hirschprung disease. Without further ado, this is the colorectal quiz. It would really suck if one time we do this and we don't record it. That's Doctor Jason Fisher, pediatric colorectal surgeon at Cincinnati Children's Hospital. Don't worry, boss, we recorded this episode. Well, welcome everyone back to our colorectal quizzes. I'm glad you're joining us because we were afraid you were gonna run away. I'm actually not convinced that it isn't just the two of us talking right now, but that's Doctor Mark Levitt, pediatric. Colorectal surgeon at Children's National. And here is the first case. We have a 10 month old with a delayed diagnosis of Hirschsprung's presented initially to the emergency room with severe abdominal distension, severe constipation, requiring cleanouts with MiraLax, but constipation remains unchanged. The pediatric surgery team was consulted and a contrast enema was obtained. No other past medical history other than this child had a failure to pass meconium at birth, secondary to a meconium plug. At that time, no suction rectal biopsy was performed during the neonatal period, and here we are now with a 10 month old, uh, suffering from significant constipation. If you're thinking to yourself, how could you miss this diagnosis of Hirschprung's? Well, think about how many kids present to their pediatrician with constipation. So why is this one different? This one has a bit of a nuance to it. The distention is significant enough to bring the kid to the ER and this history of a meconium plug. Pediatricians and gastroenterologists who see such a patient should wonder whether this is in fact uh Hirschprung's case. I was gonna ask Mark, this is, that made me pause as well. When you hear about that, and you're taking care of a patient, if you're uh uh on service in the NICU, Do you further investigate a little further? Yeah, so, I mean, let's talk about this baby as a newborn with a meconium plug that successfully passes. I would biopsy for Hirschprung's because 10% of those patients have Hirschprung's, and you do not want to have that kid limp along for the next few months, constipated, not eating well, distended, and in fact you missed the diagnosis of Hirschprung's. the other really, thank you. The real other really interesting thing about the contrast study in meconium plug is there's a meconium plug. It sounds like, yeah, duh, but I mean, think about it, this kid has Hirschprung's, so when they got that initial contrast rectal enema, it looks like a meconium plug, but really at that point, was a segment of Hirschprung's. So then after they passed the meconium, it's not a bad idea to get another contrast image, and then that will look like, oh yeah, obviously this is Hirschbprung's. But, yes, I agree, we should keep a broad differential. Amanda, there are many conditions associated with failure to pass meconium in the newborn. OK, which one's most common? Hirschprung's disease, meconium plug syndrome, meconium ileus, anal rectal malformation. OK, what are some rare ones? Small left colon syndrome, hypothyroidism, any opiates in the newborn system, any mag sulfate if the mom had preterm labor that they were trying to control. And then of course consider milk protein allergy. This last one, get ready to Google, microcolon intestinal hypoparistalsis syndrome. We already spoiled the ending for this one. I mean, this kid has Hirschsprung's, but in that time when you're doing the workup, the child should receive a rectal biopsy to rule it out. Even though Doctor Levitt and Doctor Fisher said that 9 out of 10 times it's. Gonna be negative, you probably should do it if it's high on your differential because you didn't miss the one that had Hirschprung's disease. Let's, let's take a look at the image. You got it. Let's do it. So here it is. If you're in the stay current app, scroll down under the media player, open up that first image. That's the water-soluble contrast enema at birth. Filling the entire colon. In an AP and lateral view, and hopefully you'll be able to see that on your app. But if they don't have the app, Amanda, how does it look? The colon is amorphous and very cylindrical, and there's a lot of air within the small bowel. And given these findings and no passage of meconium. Seeing this image, I can see how it might have been read as normal or just meconium plug. But this does not look right to me. It does not look like Hirschbrung's on the bottom. I guess hindsight is 20/20, no pun intended there, but a rectal biopsy should have been performed. OK, so we missed our chance of a newborn period, but remember, this patient is, again, presenting at 10 months old, in the emergency department, abdominal distention, constipation. Next to that first image is gonna be an X-ray image at the 10-month time point in the ED. We have an X-ray demonstrating dilated bowel, um, and it's abnormal, and then we have a contrast enema. That's the next picture that we're giving you in the app. Open it up, take a look, you'll see it's pretty suss. He had a significant amount of a stool burden within his entire colon. And the colon remained very cylindrical. Colon that is amorphous appearing. Uh, there's areas of irregularity along the wall. Yes, but the one thing you have to say is there's no narrowing at the um rectum compared to the sigmoid, so I could see how this would be called um normal, to be honest. What he's getting at is that this doesn't look like the classic rectosigmoid Hirschprung. These patients with total colonic. are gonna act a little bit differently, both from their imaging studies and in their presentation. So now, we want to get a biopsy, we want a definitive diagnosis, but We got a couple of options. How do you make a decision between rectal biopsy in the operating room or suction rectal biopsy at the bedside? What's your age cutoff? My cutoff is around 6 months when I switch from suction rectal biopsy to full thickness biopsy. I think there's a discussion there, and you could argue either way. Mark, I'm curious what you would do. At 10 months, I need a good diagnosis. I would probably do a full thickness biopsy at this point. Yeah, I, I agree. When I, I really, I mean, suction biopsies are great except when you don't have a definitive diagnosis and then you have to do something more definitive and then you need to go to the OR and do a formal biopsy. I think in this case, with this history, I would really want a good diagnosis, so I agree. Now, spoiler alert, this patient's going to the OR cause this is a surgical podcast, but before you do that, you have to make sure you try to get rectal biopsies pre-operatively. Um, they then went to the OR and performed, uh, biopsies. And an ileostomy. We've talked about this before, but what did the pathology show? They found no ganglion cells on frozen section. So some questions. Number 1, do you make the call Hirschbrungs or no Hirschprungs on frozen section? And 2, what do you think about the loop ileostomy? If the patient is doing well and able to be managed with irrigations, would love to have my final diagnosis of Hirschprung's disease. Through the rectal biopsy before entering the abdomen. Conversely, if the patient is not doing well with the irrigations, it kind of forces your hand, you have to give the patient a procedure to relieve that pressure, like an ileostomy or a laven colostomy. Avoid loops because Doctor Fisher and Doctor Levitt say you're probably gonna spill some stool into a non-functional part of the colon. Then, once you go to the operating room, I typically would biopsy at standard places, the rectosigmoid, maybe proximal sigmoid around the left colon, some uh biopsy along the sple uh transverse colon, and then a biopsy along the right colon or a hepatic flex area. Now, oftentimes, you can see a transition zone with your own eyes while you're in the operating room, whether it's open or with a laparoscope. In those cases where you don't, that's when Doctor Fisher and Doctor Levitt recommend, maybe you should get some more biopsies in other places like what Doctor Fisher was saying. Yeah, and I think that's an important point. I think what happened here is they confirmed Hirschsprung's disease. They went in with the laparoscope, they started their biopsies, they couldn't find any ganglion cells anywhere, and they decided to quit, not do anything definitive, and do a loop ileostomy. Now, that is totally acceptable. Don't give this patient a definitive procedure if you just have frozen section and you're questionable about that frozen section. You can always wait for pathology. You can always come back another day to do the pull through if you need to. Now let's talk about total Kalana Hirshrung. So this is less, a little bit less of a mystery right now, but obviously there's still a fair bit of controversy to talk about what, what needs to be done going forward. But we're out of time. So to hear about the next steps, you're gonna have to wait until next week when we finish this topic out. So, Amanda, help me out here. Let's summarize the case. So in summary, this is a 10 month old male who failed to pass meconium at birth and presented with severe abdominal distension and constipation to the emergency room uh with a workup at 10 months of age, uh, he was found to have a rectal biopsy. Confirming Hirschshrug's disease and underwent colonic mapping and a diverting ileostomy. Beautiful, great job. Now, we're not gonna leave you hanging completely. I mean, we're still gonna give you the weekly joke. Jason, did you hear um that 2 antennas, 2 antennas, believe it or not, got married. That's interesting. Yeah. The ceremony was great, but the reception was amazing. Oh boy, oh boy, wow, wow. So there you have it, the workup and initial management for a patient with suspected total colonic Hirschprung disease. Tune in next week to see what happened in this baby with total colonic Hirschprungs. If you need something to do while you're waiting this week, we got a lot of content in the app. Make sure to download the Stay Current Pediatric Surgery app. It's in the Apple App Store, it's in the Google Play Store, but until then, I'm Rod Gerardo, and again, I'm Amanda Jensen from Cincinnati Children's. And remember, knowledge should be free.

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