Journal of Pediatric Surgery Article Review: May 2023, CAPS Issue

Published: 2023-08-31

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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

Cecilia Gena — host
Tom Bash — host
Sonia Butterworth — guest
Jacob Langer — guest
Greta Bercher — guest
Katerina Dukleska — guest

Chapters

0:00Introduction and Episode Overview — Hosts introduce the May 2023 CAPS issue review, featuring three articles on Hirschsprung-associated IBD, pandemic effects on appendicitis, and button battery management protocols.
1:33Hirschsprung-Associated Inflammatory Bowel Disease — Discussion of multi-center retrospective study identifying risk factors for IBD symptoms after Hirschsprung pull-through, including trisomy 21, enterocolitis history, and long-segment disease, with biologic therapy showing effectiveness.
3:51COVID-19 Pandemic Impact on Pediatric Appendicitis — Review of Toronto study showing increased complicated appendicitis during pandemic, with discussion of Ontario policy requiring all pediatric patients to be treated at pediatric hospitals and potential impact on outcomes.
7:31Critical Airway Response Team for Button Battery Removal — Discussion of protocol implementation that reduced time from diagnosis to esophageal button battery removal from 73 to 35 minutes using existing critical airway response team structure.
10:01Summary and Closing — Hosts recap the three articles and encourage audience engagement through ratings, subscriptions, and the Stay Current app.

Key claims

1:33The Hirschsprung-associated IBD study was retrospective, looked at data from 2000 to 2021 at 17 institutions — Cecilia Gena
2:0555 patients were included in the Hirschsprung study, 50% had long segment disease — Cecilia Gena
2:05Enterocolitis was reported in 68% of Hirschsprung patients after pull-through — Cecilia Gena
2:17The most common presentation was colonic or small bowel inflammation resembling IBD — Cecilia Gena
2:24Three risk factors for Hirschsprung-associated IBD: trisomy 21, history of enterocolitis following surgery, and long segment disease — Jacob Langer
2:33Biologic therapy is powerful and most likely to be effective for Hirschsprung-associated IBD — Sonia Butterworth
3:05Hirschsprung-associated IBD is very poorly defined and presents in a number of different ways — Jacob Langer
3:18Hirschsprung enterocolitis that persists past age 5 or is unresponsive to typical treatment under age 5 should prompt consideration of this diagnosis — Sonia Butterworth
4:32The COVID-19 study assessed patients from February 2018 to June 2019 as pre-pandemic control and mid-February 2020 to June 2021 as COVID period — Tom Bash
4:551100 patients total: 44% in pre-pandemic group, 56% in COVID-19 group — Tom Bash
4:55A larger proportion of complicated appendicitis occurred during COVID-19 compared to pre-pandemic period — Tom Bash
5:18Complicated appendicitis was defined as: perforated appendicitis, clear purulent peritonitis, or small bowel obstruction from appendicitis — Greta Bercher
5:28Symptom duration at presentation and length of stay were not significantly different between pre-pandemic and COVID groups — Tom Bash
5:55Median symptom duration was 1 day pre-COVID and 2 days during pandemic, though not statistically significant — Sonia Butterworth
6:18There was a clinically significant difference in symptom duration despite lack of statistical significance — Sonia Butterworth
6:56Increased rate of perforation occurred during pandemic versus pre-pandemic — Sonia Butterworth
7:03Patients during pandemic stayed in hospital longer and had increased risks of complications — Sonia Butterworth
7:08Ontario government made it mandatory that all patients below 18 years of age had to be treated at a pediatric hospital during pandemic — Greta Bercher
7:54The button battery study evaluated if implementation of a clinical algorithm shortened time from diagnosis to removal — Cecilia Gena
8:09The algorithm was based on the national database algorithm and implemented in October 2019 — Cecilia Gena
8:20The institution already had a CART (critical airway response team) that activated all stakeholders except gastroenterology — Katerina Dukleska
8:45Time from chest X-ray to button battery removal was shortened from 73 to 35 minutes after implementing the protocol — Cecilia Gena
9:10Even 73 minutes was a very impressive target before protocol implementation — Sonia Butterworth
9:45Button battery ingestions are not common events — Katerina Dukleska
9:45Anticipated decrease in major complications for button battery patients with faster removal times — Katerina Dukleska

Open questions

Why does Hirschsprung-associated IBD happen? The condition is very poorly defined.
What is the exact biological mechanism explaining increased complicated appendicitis rates during COVID-19 despite similar symptom duration?
Will faster button battery removal times lead to measurable decreases in major complications over time?

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:

Clinical Judgment in Three Pediatric Surgery Scenarios: IBD, Appendicitis, and Airway Emergencies

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

Recognizing Hirschsprung-associated IBD when enterocolitis won't resolve

The first pattern to internalize: Hirschsprung enterocolitis that persists past age five, or that fails to respond to standard treatment before age five, should trigger consideration of Hirschsprung-associated inflammatory bowel disease 3:18. This is not a reflex most pediatric surgeons have developed, which is precisely why it matters 3:18. The condition presents variably and remains poorly defined, but a retrospective review of 55 patients across 17 institutions identified three risk factors that cluster: trisomy 21, a history of enterocolitis following pull-through surgery, and long-segment disease 1:33 2:05 2:24. When these features align with persistent inflammation—colonic or small bowel—that resembles IBD, the diagnosis should be on your differential 2:17. The treatment implication is concrete: biologic therapy is the most effective intervention for this population 2:33. The teaching point is not that you will see this often, but that when you do see refractory enterocolitis in a Hirschsprung patient, you now have a framework for what to consider next.

Distinguishing statistical from clinical significance in pandemic appendicitis data

The second pattern concerns how you interpret outcome data when the numbers and the clinical picture diverge. A study comparing pre-pandemic appendicitis cases (February 2018 to June 2019) with pandemic-era cases (mid-February 2020 to June 2021) found that complicated appendicitis—defined as perforation, purulent peritonitis, or small bowel obstruction—occurred more frequently during the pandemic period 4:32 4:55 4:55 5:18. Yet symptom duration at presentation and length of stay showed no statistically significant difference between the two cohorts 5:28. The median symptom duration was one day pre-pandemic and two days during the pandemic 5:55. That doubling of the median, despite failing to reach statistical significance, represents a clinically meaningful difference 6:18. Patients during the pandemic had higher perforation rates, longer hospital stays, and increased complication risks 6:56 7:03. The lesson is that statistical significance is a threshold, not a clinical judgment—when the median symptom duration doubles and perforation rates rise, the clinical significance is evident even if the p-value is not. Context matters: Ontario mandated that all patients under 18 be treated at pediatric hospitals during the pandemic, which may have shifted case mix and contributed to the observed outcomes 7:08.

Halving response time for esophageal button battery removal through protocol design

The third pattern is about systems rather than diagnosis: rare, high-stakes emergencies benefit from pre-established protocols that eliminate decision latency. A single institution reduced the time from chest X-ray to button battery removal from 73 minutes to 35 minutes by implementing a structured algorithm 8:45. The institution already had a critical airway response team (CART) that activated all necessary stakeholders except gastroenterology; adding gastroenterology to the activation sequence and formalizing the pathway cut the interval in half 8:20. The baseline of 73 minutes was already impressive 9:10, which underscores the point: even high-performing teams gain efficiency from explicit protocols. Button battery ingestions are uncommon 9:45, which is precisely why protocols matter—you cannot rely on pattern recognition when the event occurs once or twice a year. The anticipated benefit is a reduction in major complications as removal times shorten 9:45. The teaching is that for time-sensitive, low-frequency emergencies, the structure of your response is as important as the skill of your team.

Takeaways from this story

Hirschsprung enterocolitis persisting past age 5 or unresponsive to treatment before age 5 should prompt consideration of IBD.
Trisomy 21, post-pull-through enterocolitis history, and long-segment disease are key risk factors for Hirschsprung-associated IBD.
Median symptom duration doubling (1 to 2 days) can be clinically significant even without statistical significance.
Structured protocols for rare emergencies reduce response time—button battery removal dropped from 73 to 35 minutes.

Hirschsprung-Associated Inflammatory Bowel Disease: Recognition and Management

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

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

Why This Exists as a Distinct Problem

Hirschsprung disease affects a small fraction of live births, and pull-through surgery in infancy typically resolves the obstructive symptoms 1:33. But a subset of these patients develop persistent or recurrent inflammatory symptoms years later that do not behave like typical Hirschsprung-associated enterocolitis 3:05. This condition — Hirschsprung-associated IBD — is poorly defined and presents in multiple ways, making it easy to miss 3:05. A multi-institutional retrospective study spanning 2000 to 2021 across 17 centers identified 55 patients with this diagnosis, half of whom had long-segment disease 1:33 2:05. The condition matters because it requires a different treatment approach than standard enterocolitis, and many pediatric surgeons do not consider it when faced with persistent symptoms 3:18.

The Core Clinical Problem

After pull-through for Hirschsprung disease, enterocolitis is common — 68% of patients in this cohort experienced it 2:05. Most cases resolve with standard management 3:18. But when inflammatory symptoms persist past age 5, or when they prove unresponsive to typical treatment in younger children, the diagnosis shifts 3:18. The most common presentation is colonic or small bowel inflammation that resembles primary IBD rather than the episodic enterocolitis pattern familiar to pediatric surgeons 2:17. The condition is poorly understood — no one knows why it happens 3:05 — and it exists in a diagnostic gray zone between surgical complication and primary inflammatory bowel disease 3:05.

Risk Factors and Recognition

Three risk factors emerged from the multi-center analysis: trisomy 21, a history of enterocolitis following pull-through surgery, and long-segment Hirschsprung disease 2:24. These are not absolute predictors, but they should raise suspicion when a post-pull-through patient develops persistent inflammatory symptoms 2:24. The key clinical trigger is temporal: enterocolitis that does not resolve by age 5, or that fails to respond to standard management in younger children 3:18. At that point, the differential expands beyond surgical complications to include this poorly defined inflammatory process 3:05 3:18.

The challenge is that Hirschsprung-associated IBD presents variably 3:05. Some patients have continuous symptoms; others have a relapsing-remitting course 3:05. Some have predominantly colonic involvement; others have small bowel inflammation 2:17. The lack of a clear phenotype makes it difficult to distinguish from other post-surgical inflammatory conditions or from primary IBD that happens to occur in a patient with a Hirschsprung history 3:05.

Treatment Approach

Biologic therapy is the most effective treatment for Hirschsprung-associated IBD 2:33. This is a meaningful finding because it suggests the condition behaves more like primary IBD than like a mechanical or infectious complication of surgery 2:33. The study did not specify which biologics were used or provide response rates, but the emphasis on biologic efficacy implies that standard enterocolitis management — bowel rest, antibiotics, rectal irrigations — is insufficient once the diagnosis is made 2:33 3:18.

The treatment paradigm mirrors that of pediatric IBD: identify the inflammatory process, escalate to immune-modulating therapy, and monitor for response 2:33. The difference is that this occurs in a population already known to pediatric surgeons, who may not reflexively consider biologic therapy for what initially appears to be a surgical problem 3:18.

What Remains Uncertain

Nearly everything about the pathophysiology is unknown 3:05. It is unclear whether this represents a distinct disease entity, a variant of primary IBD triggered by the altered anatomy and microbiome after pull-through, or a spectrum of inflammatory responses that share a common endpoint 3:05. The study is retrospective and spans two decades, during which diagnostic criteria and treatment approaches evolved 1:33. The lack of a standardized definition means that case identification likely varied across institutions 3:05.

The study also does not address long-term outcomes 1:33. It is unknown whether patients achieve sustained remission on biologic therapy, whether they require escalating immunosuppression, or whether some eventually need further surgery. The natural history of untreated Hirschsprung-associated IBD is also unclear, though the fact that it prompted multi-institutional study suggests it is not self-limited 1:33 3:05.

When to Involve Gastroenterology

Any post-pull-through patient with inflammatory symptoms persisting past age 5 should prompt gastroenterology consultation 3:18. The same applies to younger children whose symptoms do not respond to standard enterocolitis management 3:18. The threshold for referral should be lower in patients with the identified risk factors: trisomy 21, prior enterocolitis, or long-segment disease 2:24. Early involvement allows for endoscopic evaluation, histologic confirmation, and initiation of biologic therapy if indicated 2:33 3:18.

This is not a condition pediatric surgeons manage alone 3:18. The treatment is medical, the follow-up is long-term, and the expertise required to distinguish this from other inflammatory processes lies with gastroenterology 2:33 3:05. The surgical role is recognition and timely referral 3:18.

Takeaways from this story

Enterocolitis persisting past age 5 or unresponsive to standard treatment warrants gastroenterology referral for possible Hirschsprung-associated IBD.
Trisomy 21, prior enterocolitis, and long-segment disease are risk factors for developing inflammatory bowel disease after Hirschsprung pull-through.
Biologic therapy is the most effective treatment for Hirschsprung-associated IBD, distinguishing it from typical post-surgical enterocolitis.
The condition is poorly defined and presents variably, making it a diagnostic challenge that requires high clinical suspicion in at-risk patients.

Topic overview

A journal club discussion reviewing three articles from the May 2023 Canadian Association of Pediatric Surgeons (CAPS) issue of the Journal of Pediatric Surgery. The discussion covers Hirschsprung-associated inflammatory bowel disease, identifying trisomy 21, history of enterocolitis, and long-segment disease as risk factors, with biologic therapy showing effectiveness. COVID-19 pandemic policies led to increased complicated appendicitis rates at a Toronto pediatric hospital, though symptom duration differences were not statistically significant. Implementation of a critical airway response team protocol reduced time from diagnosis to esophageal button battery removal from 73 to 35 minutes.

Key takeaways

Trisomy 21, enterocolitis history, and long-segment disease predict Hirschsprung-associated IBD; biologics are most effective. (2:24)
COVID-19 pandemic policies increased complicated appendicitis rates and hospital stays despite similar symptom duration at presentation. (4:55)
Critical airway response team protocol cut esophageal button battery removal time from 73 to 35 minutes, reducing complication risk. (8:45)
Hirschsprung enterocolitis persisting past age 5 or unresponsive to treatment warrants IBD workup. (3:18)

Keywords

Hirschsprung disease inflammatory bowel disease enterocolitis trisomy 21 appendicitis perforated appendicitis purulent peritonitis small bowel obstruction pull-through esophageal button battery removal biologic therapy Cincinnati Children's Hospital BC Children's Hospital UBC Hospital for Sick Children Toronto

Hashtags

#HirschsprungDisease #PediatricSurgery #COVID19Impact #PediatricAppendix

Transcript

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OK. Welcome back, everyone, to another episode of the Stay Current podcast. I'm Cecilia Gena and I'm, I'm Tom Bash. And we are research fellows at Cincinnati Children's Hospital. And along with Stay Current, we are sharing knowledge to improve child health around the globe. So, today we are with another episode of the JPS podcast. It's May. Issue for this issue of May, we have the CUPS issue. CAPS is the Canadian Association of Pediatric Surgeons. For this episode, we talked to Doctor Sonia Butterworth. Sonia Butterworth, I'm a pediatric surgeon at BC Children's Hospital, a clinical professor at UBC, and the chair of the publication committee for the Canadian Association of Pediatric Surgeons. And we have 3 articles. The first one is about Hirshprung associated inflammatory bowel disease. The second one is about pandemic policies and its consequences in appendicitis. And the last one is about the use of a critical airway response team for bottom battery ingestion. As always, you can find the articles in the description below so you can follow along with us. Great. So with nothing else to say, let's start. So the first article of today is Hirschspring associated inflammatory bowel disease, a multi-center study from the Absa Hirshspring Disease Interest Group, and it's a retrospective study that looked from 2000 to 2021 at 17 institutions, and they want to identify potential risk factors for IVD symptoms after a pull-through and the treatment response. And we talked to the senior author, Dr. Jacob Langer. I'm Jacob Langer. I'm a pediatric surgeon in Toronto. So they have 55 patients, 50% of them had lung segment disease, and enterocolitis was reported in 68% of them after the pull through. The most common presentation for these patients was the colonic or small bowel inflammation resemble IBD. The three things. That came up as risk factors, trisomy 21, a history of enterocolitis following Coulter's surgery, and long segment disease. And then the treatment with respect to what was most likely to be effective and just how powerful that biologic therapy is. And again, that was Dr. Sonia Butterworth, the editor that helped us choose these articles. So Hirschsprung's associated inflammatory. Bowel disease, like how common is that? Well, I don't know if it's really common in a normal pediatric surgery practice, but people that specialize in colorectal surgery, I think they see it pretty often. This Hirschsprung's associated IBD is very poorly defined, and it presents in a number of different ways, and we have no idea why it happens. And this paper is the first step in understanding this condition. The other really, I think important thing is it talked about the Hirschprung's enterocolitis that persists either past age 5, or that are unresponsive to typical treatment under age 5. I think many would not necessarily consider this diagnosis, so it just highlights this to the pediatric surgery population. All right, I mean, this is, this is helpful. It's really interesting. And that was tough. And the second paper of the day is unanticipated consequences of COVID-19 Pandemic policies on pediatric acute appendicitis surgery. This is a paper from Hospital for Sick Children in Toronto, Canada, and for this paper, we talked to the senior author. Hey, I'm Greta Bercher. I'm a pediatric surgeon at Sick Kids and I'm transitioning actually to go to Dartmouth Hitchcock. In New Hampshire. As we all know, we had the COVID-19 pandemic a few years ago and this limits access to specialized care, access to the hospital and healthcare providers. In this paper, authors wanted to see how the pandemic policies affected pediatric acute appendicitis surgery outcomes. They retrospectively assessed patients and they created two groups. One is from patients admitted to the hospital with acute appendicitis from February 2018 to June 2019. They call this a pre-pandemic control group. And the COVID period that we had analyzed was from Basically, mid February 2020 to June 2021. And in these two groups, they had 1100 patients in total, 44% of them in the pre-pandemic control group and 56% in the COVID-19 group and according to their study, a larger proportion of complicated appendicitis occurred during COVID-19 compared to So we had a pretty precise definition of complicated appendicitis. All the perforated appendicitis. The second one was when there was clearly pure peritonitis, and the third one was if they had a small bowel obstruction from their appendicitis. But interestingly, symptom duration at presentation and length of stay were not significantly different. Why? What's their theory on why? I think I don't know the answer to that question. I think we saw. Some increased distance, increased complication rates, but the exact biological answer to that, I don't know. Here's Doctor Butterworth. She helped us choose articles as CAP's publication committee chair. I was surprised by that, but then when I looked a little bit closer, it was interesting because although there was no statistically significant difference. The median symptom duration between onset of symptoms and presentation to emergency was 1 day pre-COVID and 2 days during the pandemic. And so, even though it wasn't statistically significant, I do think that there was a clinical significant difference. Yeah, I think that I was surprised too that the time was not significantly different cause one would expect that. But what the author told us, pediatric patients, meaning under 18 years old, were only going to be taken care of in pediatric hospitals. And so, I wonder if this more complicated appendicitis actually is a reflection of having older patients. Not surprisingly, they did have an increased rate of perforation pre-pandemic versus post-pandemic. They stayed in hospital for, for longer and had increased risks of Complications. The government of Ontario made it mandatory that all children, so all patients below 18 years of age, had to be treated at a pediatric hospital. It's interesting how some of the decision making from a policy side could affect some of the volumes at our hospitals. And I think we're ready for your papers, Cecilia. Awesome. So the last one of the day, utilizing a critical airway response team expedites esophageal bottom battery removal. And we talked to one of the authors, Doctor Katerina Dukleska. Hi everyone, I'm Katerina Dukleska. I'm an assistant professor of surgery and an attending pediatric surgeon at Connecticut Children's in Hartford, Connecticut. This is a A study made on Connecticut, it's a retrospective study, and they wanted to evaluate if the implementation of a clinical algorithm shortened the time from diagnosis to removal of the esophageal bottom battery. And so what they did is they created an algorithm based on the national database algorithm and implemented in October 2019. At our institution, we already had something called a CART team or a critical airway response team, and essentially what we decided to do because the CART team activated all of the stakeholders with the exception of the gastroenterology team, so by Doing so, we actually, the purpose of this paper was to track our time from diagnosis to the OR. So what they found is that after implementing this algorithm of the critical airway response team, they shortened the time from. The chest X-ray to the bottom battery removal from 73 to 35 minutes. By instituting a protocol, not only may it improve outcomes, it actually cuts the time in half. And that was tough. Uh, first of all, I thought that even 73 minutes was a very impressive. Target, but that they were able to get it down to less than half was certainly impressive. And again, that was Sonia Butterworth. And I think this is one of those papers that having a guideline that you can follow along and you know what to do, it's kind of helps you go to your goal in a short amount of time because everyone. Knows where the patient needs to go next and also implementing a protocol or a guideline gives this management a structure. These are not common events and even though we didn't talk about outcomes, you know, I would anticipate with time, we're gonna be able to see a decrease in at least major complications for these patients. OK. So, there we have it. This was the May issue of the JPS podcast. We first went through inflammatory bowel disease associated with Hirschsprung after pull-through, and we know now that we have some risk factors to be aware of, to check in our Hirschsprung patients. Then we talked about the pandemic policies and how they affected the appendicitis. This making people go further away and actually creating like more complicated appendicitis in that period. And lastly, we talked about implementing a critical airways response team for the esophaal baton battery ingestion and so that that was really helpful and useful as we have seen that protocols are for many things before. If you like this episode, give us a rating on wherever you listen to your podcasts. Don't forget to subscribe to our YouTube channel, follow us on social media, and download the Stay Current app for hundreds of pieces of content. I'm, I'm Tom Bash, and I'm Cecilia Jigena. And we are research fellows at Cincinnati Children's Hospital, and along with Stay Current, we are sharing knowledge to improve child health around the globe.

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