Update Course Rewind: Pediatric Colorectal Consortium 2021

Published: 2022-04-28

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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
Ellen Ancisco — host
Rebecca Rentia — guest
Caitlin Smith — guest
Speaker 5

Chapters

0:00Introduction and Anal Dilations After PSARP — Hosts introduce the 2021 PCPLC update session with Drs. Rentia and Smith. First case discusses a 3-month-old with ARM post-PSARP, leading to review of evidence that anal dilations may not be needed, based on a single-institution RCT showing no difference in stricture rates at 12 months.
4:14Timing of Repair for Perineal and Vestibular Fistulas — Case of newborn female with anterior anus. Discussion of early versus late repair timing for perineal and rectovestibular fistulas, reviewing two studies showing no difference in 30-day complications. Practical considerations include infant feeding type and timing relative to introduction of solid foods.
6:57Hirschsprung Disease: Long Segment and Total Colonic — Cases addressing total colonic Hirschsprung disease management. Review of literature showing shift toward earlier pull-through (around 5 months). Discussion of hypermotility protocol and importance of stool management strategies. Emphasis on avoiding delayed repair to prevent anal sphincter spasm and pelvic disease.
9:49Bowel Management and Disparities in ARM — Case of 5-year-old with rectoprostatic malformation and daily soiling. Review of PCPLC data on bowel management strategies showing only 40% of ARM patients aged 5-12 are toilet trained, with majority requiring enemas. Discussion of disparities study showing public insurance associated with decreased urinary incontinence rates.
12:41Cloacal Reconstruction and Conclusion — Technical considerations in cloacal reconstruction, emphasizing importance of urethral length preservation (minimum 1.5 cm needed to avoid incontinence). Role of 3D cloacography for preoperative planning. Session concludes with summary of key points covered.

Key claims

1:10A single institution prospective randomized control trial found that anal dilations following PSARP may not be needed — Rebecca Rentia
1:31There is literature suggesting a component of psychosocial, psychologic dissociation in children on later testing related to dilations — Ellen Ancisco
1:37In the dilation study, length of follow-up was 12 months, PSARP had to be performed in a child under 24 months of age, it was primary surgery, and excluded Cloaca as a diagnosis — Rebecca Rentia
1:50The average PSARP was performed at 5 months in the study — Rebecca Rentia
1:52A stricture was defined as a Hagar dilator size of less than 10 — Rebecca Rentia
1:57The standard Hagar dilator size for a newborn is about size 12 — Rod
2:16There were 25 children in the dilation study and the types of malformations were evenly distributed — Rod
2:26Strictures were non-significant between both groups in the dilation study — Rebecca Rentia
2:26A Heineke-Mikolliz anoplasty is where a longitudinal incision is made toward the anus and then closed transversely to widen the diameter — Rebecca Rentia
2:55The number of strictures, number who needed anoplasties, and number of redo operations was the same between the dilation and no-dilation groups — Ellen Ancisco
3:04The number of patients who had rectal prolapse is consistent with the literature — Ellen Ancisco
4:04About 5 to 8% of patients require a strictureplasty at the two-month period — Rebecca Rentia
5:20A study looked at 30-day outcomes for patients with ARM with perineal or rectovestibular fistulas who underwent repair, divided between early (before 6 days old) and late repair (6-8 weeks) — Ellen Ancisco
5:31There were 291 patients in the early vs late repair study: 66 underwent early repair and 231 underwent late repair — Ellen Ancisco
5:4030-day complications are not statistically different between early and late repair groups for perineal and rectovestibular fistulas — Caitlin Smith
6:22For neonates and infants, dilations are really well tolerated for patients up to even several months old — Caitlin Smith
6:32Formula-fed infants who need caloric concentration have thicker stools, which might push toward earlier repair — Caitlin Smith
6:39For breastfed infants, repair can be delayed until 2 to 3 months — Caitlin Smith
6:47You want to get repair done before infants start solids because that can make the dilation strategy at home much more difficult — Caitlin Smith
7:11Literature shows a shift toward slightly earlier pull-through for Hirschsprung disease — Rebecca Rentia
7:27Long segment Hirschsprung is defined as any disease proximal to the rectosigmoid colon for the majority of articles reviewed — Rebecca Rentia
7:42A contrast study itself is very inaccurate for Hirschsprung disease and colonic mapping needs to be performed to determine the level of the transition zone — Rebecca Rentia
7:51There was no superior operation or more common operation performed for long segment Hirschsprung, although Duhamel and Swenson-Soave were the top operations — Rebecca Rentia
8:13There are no new novel surgical techniques for Hirschsprung disease over the past several years — Rod
8:21There is potential for stem cell therapy for Hirschsprung disease but it is still in its infancy — Rebecca Rentia
8:35An early operation for total colonic Hirschsprung is possible around the age of 5 months old — Rebecca Rentia
8:49If a child with an ileostomy is adequately prepared and the family can participate in learning to thicken up stool, they can have a pull-through that does not result in complete perineal skin breakdown — Rebecca Rentia
9:34If you wait way too long to do a pull-through especially for total colonic Hirschsprung, patients will have horrible anal sphincter spasm and potentially horrible pelvic disease that makes keeping a pull-through really challenging — Rebecca Rentia
10:36A PCPLC study looked at bowel management strategies in children with ARM, examining 624 patients with varying diagnoses of severity — Caitlin Smith
11:14The majority of ARM patients had constipation as their primary complaint and only 40% were toilet trained — Caitlin Smith
10:55Even mild and moderate ARM patients in the 5 to 12 year old group need to rely on enemas and lots of other bowel management strategies to stay clean when heading into school age — Caitlin Smith
11:38For Hirschsprung timing study, all infants had to be diagnosed under 1 month of age with primary pull-throughs less than 31 days or greater than 31 days — Rebecca Rentia
11:46Preoperative enterocolitis was the same between early and late pull-through groups for Hirschsprung — Rebecca Rentia
11:51Post-operative enterocolitis was the same between early and late pull-through groups and transition zone was the marker if a child needed to be treated for constipation — Rebecca Rentia
12:05Non-clinical factors including race, ethnicity, and insurance status are associated regardless of clinical variables with outcomes in colorectal patients — Caitlin Smith
12:21A PCPLC consortium study of 525 ARM patients found public insurance was associated with decreased rates of urinary incontinence — Caitlin Smith
13:26The urethral length of about 2.5 centimeters has been measured in a lot of VCUGs of normal females — Rebecca Rentia
13:41About 1.5 centimeters of urethral length is needed in cloacal reconstruction — Rebecca Rentia
13:44If a urethra that is too short is pulled past the bladder neck, there is a risk for incontinence — Rebecca Rentia
13:55A short vagina, even in an otherwise shorter common channel operation, may require a vaginal replacement — Rebecca Rentia
14:03Rotational fluoroscopy and 3D reconstructions are key to being able to make reliable measurements for anatomy in cloacal cases — Rebecca Rentia

Cases discussed

0:443-month-old with anorectal malformation with rectal bladder neck fistula, presenting 2 weeks after laparoscopic-assisted anorectoplasty with colostomy and mucous fistula
4:16Newborn female at 39 weeks, 2.5 kg, with anterior anus, short perineal body, anal opening anterior to muscle complex but posterior to labial folds
7:023-month-old male with total colonic Hirschsprung disease and end ileostomy
9:495-year-old boy with rectoprostatic malformation, history of repair in infancy, tethered cord repair, presenting with daily soiling

Open questions

Whether race and ethnic disparities in ARM outcomes exist beyond what is captured at specialty centers, particularly for patients who cannot afford to travel to consortium centers
The role of stem cell therapy in Hirschsprung disease treatment, which is still in early development
Whether rectal prolapse after PSARP will resolve spontaneously or require intervention

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.

Topic overview

A panel discussion reviewing key clinical updates from the 2021 Pediatric Colorectal Consortium, covering management of anorectal malformations and Hirschsprung disease. The session addresses whether anal dilations are necessary after posterior sagittal anorectoplasty, with a randomized trial showing no difference in stricture rates between dilation and no-dilation groups at 12 months. Timing of surgical repair is discussed for both anorectal malformations (early vs. late repair showing equivalent 30-day outcomes) and Hirschsprung disease (shift toward earlier pull-through around 5 months for total colonic disease). Additional topics include bowel management strategies, with data showing only 40% of ARM patients aged 5-12 are toilet trained, and technical considerations in cloacal reconstruction including the importance of preserving adequate urethral length (minimum 1.5 cm needed).

Key takeaways

Anal dilations after PSARP may be unnecessary—RCT showed no difference in stricture rates at 12 months between groups. (1:10)
Early vs late ARM repair (perineal/vestibular fistulas) shows equivalent 30-day outcomes; timing can be individualized. (5:20)
Only 40% of ARM patients aged 5-12 are toilet trained; even mild cases require bowel management strategies at school age. (10:55)
Total colonic Hirschsprung pull-through feasible at ~5 months if family trained in stool management to prevent breakdown. (8:35)
3D fluoroscopy critical in cloacal cases—urethral length ≥1.5cm needed to avoid incontinence when mobilizing past bladder neck. (13:41)

Keywords

anorectal malformation rectal bladder neck fistula tethered cord Hirschsprung disease total colonic Hirschsprung disease rectoprostatic malformation perineal fistula rectovestibular fistula cloaca anal stricture rectal prolapse enterocolitis constipation fecal incontinence urinary incontinence

Hashtags

#PediatricColorectal #AnorectalMalformation #PediatricSurgery #PSARP

Transcript

Click "Show Transcript" to view the full text (14783 characters)

Did you miss our 2021 pediatric surgery update course? Well, don't worry, we're gonna give you our favorite sessions right here on this podcast. I'm Rod and I'm Ellen Ancisco. We're research fellows at Cincinnati Children's Hospital Medical Center. This was a super important session. It's on colorectal surgery. All right, so I'm gonna turn things over to Doctors Rebecca Rantia and Caitlin Smith. They're gonna explain the consortium. I'm Rebecca Rentia. I'm at Children's Mercy in Kansas City, and I do quite a bit of pediatric colorectal surgery, and that's one of my interests in concentrations. And I'm joined by Doctor Caitlin Smith. I'm Caitlin Smith. Um, I'm at Seattle Children's and I'm the program director of the reconstructive Pelvic medicine Program here. So here's the first case. You see a 3 month old with a history of an anorectal malformation with a rectal bladder neck fistula. He presents 2 weeks after laparoscopic-assisted anal ectoplasty. a colostomy and a mucous fistula. What's your next step in management? And so our first hot topic is anal dilations following posterior sagittal anorectoplasty or the PSA. As always, scroll down under the media player. You can find the links to the articles below. And what this study found in a single institution prospective randomized control trial is that they may not be needed. And so, we know that there's a lot of psychologic trauma that goes. Um, with families having to perform the dilations on their newborn children or having to perform them on a young child. And there's literature suggesting that there is a component of psychosocial, psychologic dissociation in a child on later testing. The length of follow-up in this study was 12 months. The PSARP um had to be performed in a child under 24 months of age. It was a primary surgery, excluded Cloaca as a diagnosis. The average PSARP was performed at 5 months. And a stricture was defined as a Hagar dilator size of less than 10. So keep in mind that the standard Hagar dilator size for a newborn is going to be about size 12. So a size 10 is 2 deviations less. So they looked at primary and secondary outcomes, and one of the questions that they asked is, what was the need for re-operation and additional surgeries in each arm of the study. In this study, there were 25 children, and the types of malformations were evenly distributed. So the complexity of the children was about equal. There were strictures that were non-significant between both groups, and essentially a Heineke-Mikolliz anoplasty, which is where a longitudinal incision is made toward the anus and then it's closed transversely to widen the diameter of the circle, was able to be performed. Some of the few that had To be done for stricture, did have to be done under separate general anesthesia, but for children who had a colostomy, that stricture was able to be taken care of at the time of colostomy to be done. So what we see in this study is that the number of strictures is the same between groups, the number who needed anoplasties is the same, and the number of redo operations is the same between the two groups. And the number of patients who had rectal pleural lapse is consistent with the literature. So there is a really good case here to abandon doing dilations postoperatively for children with anorectal malformations. I would say that currently in practice, I do size the anus at 2 weeks and 1 month, and I believe a lot of surgeons really still need to understand what the diameter of the anoplasty is so that stooling can um not be. Obstructed by a strictured anoplasty that's unrecognized. However, since the HM anoplasty is an option, and we know that for slightly older children, dilations really are even more traumatic than for neonates, and you're concerned that you might need to put the child under a general anesthesia, that would be the only time that I would consider now initiating full dilations. What would you say the current percentage of patients who require a strictureplasty at that, at that two-month period? Probably about 5 to 8%. And there's also in there, the question always if there's prolapse, will it go away? But yeah, I would say about 5 to 8%. All right, so moving on to our next case. Let's say you had a female patient transferred to your hospital. She's 39 weeks. She is 2.5 kg. Physical exam is normal, except she has a short perineal body, and then the anal opening is actually anterior to the muscle complex. But It's still posterior to the labial folds. So then you size her up with the Hagar dilators. She's about a size 6. You get a little bit of stool output when you do that. Then you're gonna work her up with an echocardiogram. You're gonna find out she has a PDA. She has a little bit of left to right flow. And then on a renal ultrasound, she's a little bit of hydronephrosis on the left, and then on her spine, she has a conus that terminates at about L1. OK, so this infant now is in your NICU. What are you gonna do? So, I'm, I'm a fan of doing dilations in the neonatal period and having the family um be discharged as soon as possible to home. So this is a study looking at 30-day outcomes for patients with anorectal malformations with perineal or recive vestibular fistulas who underwent repair. They. Divided between early and late repair. Early to repair was defined as patients who underwent repair before they were 6 days old. Late repair was defined for patients who underwent repair between 6 and 8 weeks of age. There were 291 patients in the study, 66 underwent early repair, and 231 underwent late repair. So what were the outcomes? The ultimate conclusion was that 30-day complications are not statistically different between these two groups. Um, also looking at perineal and rectal vestibular fistulas, early was defined slightly differently as 14 days, or younger, um, and then late was defined, late repair was defined as anytime after 14 days old. 164 patients were looked at, 31 early and 133 late repairs, and also, um, the conclusion of this paper was that no difference in 30-day outcomes in terms of complications. OK, so assuming that this is a healthy baby. What do you do? Do you do the dilation, send them home? Is there an optimal age? What do you think? I think for the neonates, for the infants, the dilations are really well tolerated, um, for patients who are up to even several months old. Patients who have older repair, I avoid them in those age groups. Things that might push you to do it earlier would be a little earlier would be like if it's a formula-fed infant who needs like caloric concentration. And their stools are thicker, like you can delay for, uh, definitely for like a breastfed infant, um, until 2 to 3 months, that's fine. You really wanna get it done before they start solids also because that can make the dilation strategy at home, um, you know, much more difficult. So those are the things in terms of timing that I think of. All right, let's change gears. Let's talk about a different disease process. Let's talk about Hirschsprung disease. A 3 month old male presents to your clinic to establish surgical care. He has a history of total colonic Hirschberg disease and has an end ileostomy. When would you recommend pull through? What the literature really shows is that there's a shift that's happening to a slightly earlier pull through. OK, so what are the long-term outcomes? Do we have any novel surgical techniques and I mean, just to kind of cover the basics, how do you even define a long segment? It's any disease that's proximal to the rectosigmoid colon for the majority of articles that were reviewed. So this is a pediatric surgery Hirschprung interest group article and it discusses how to talk about Hirschprung disease with the pathology group in the operating room and in the studies. Um, and then a contrast study itself is very inaccurate, um, and colonic mapping needs to be performed to determine the level of the transition zone. There was no superior operation or more common operation that was performed, although DuML and a Swenson Suave were kind of the top operations. So a lot of this kind of depends on the pediatric colorectal surgeon you talked to. I mean, Swenson, Suave, Dumel, I mean, is there really one that is more superior to the other, more common than the other? That might vary based on the surgeon, but there are certainly no new novel surgical techniques, at least over the past several years. Years. There's a potential for stem cell therapy, but that's still in its infancy. This article is about a hypermotility and skin rash protocol for patients with total colonic Hirschprung disease. It is published in the European Journal of P Paediatric Surgery. So why is it important? It has a mixed population of children with total colonic Hirschprung's, but it outlines very clearly why an early operation for total colonic Hirschprung's is possible, and by early, I mean around the age of 5 months old, and why an operation. at a slightly earlier age than toilet training for urine is possible is because if a child with an ileostomy is adequately prepared and the family is able to participate in learning to thicken up stool, then they are able to actually have a pull-through that does not result in complete perineal skin breakdown and ultimately learn techniques that will help this child because this is one of the hardest groups to Toilet train overall. OK, so regardless of the urinary incontinence, this is total colonic Hirschprung. The, the question otherwise really becomes, um, I just always do it when the child is toilet trained for urine. I only do it at the age of 4. And I think if you wait way too long to do a pull through a special of total colonic, they will have horrible anal sphincter spasm and potentially horrible pelvic disease that makes keeping a pull through really challenging. So let's move on to case number 4. This is a 5 year old boy with a recoprostatic malformation, history of repair, but hasn't really followed since then. At that time, in infancy, he also had a tethered cord repaired. Sacred ratio is 0.5. It comes to your clinic now with daily soiling, multiple times, still in diapers, ready to go to kindergarten in the fall, but doesn't really have the sensation or verbalize when he's ready to stool. You do your exam under anesthesia and you find that there's not a stricture, and the anoplasty is actually pretty well centered. So what do you do? I think it's really um challenging and especially as they often have had surgery and not necessarily been followed long term. Um, so this, uh, study is out of the PCPLC looking at bowel management strategies in children with anorectal malformations. Um, it came out in, uh, just last year, and I think this is a really important graph because it shows what percentage of, um, you know, even mild, uh, and moderate. rectum malformation patients in this 5 to 12 year old group really need to rely on enemas and lots of other bowel management strategies to stay clean when they're heading into school age. Overall, looked at 624 patients um that were in the PCPLC registry with all with varying diagnoses of severity of anorectal malformation. Um, the majority of patients had constipation as their primary complaint, um, and only 40%, uh, were toilet trained, which I think is a really important, uh, note as well. For these patients, you really have to do long-term close follow-up, especially through the toilet training years to make sure that you don't have issues like this. Point number 5 is the timing of poultry for Hirschberg disease. All infants had to be diagnosed. Diagnosed under 1 month of age. These were primary pull-throughs less than 31 days or greater than 31 days. And what the study found was that preoperative entercolitis was the same between both groups. Post-operative enterocolitis was the same, and transition zone really was the marker if a child needed to be treated for constipation. Talking about disparities in um patients in the colorectal population. Um, obviously, we are well aware that there are non-clinical factors, race, ethnicity, and insurance status that are associated regardless of clinical variables with outcomes. This, uh, data was presented at APSA this year, um, and was recently accepted. This is another consortium study out of the PCPLC that looked at 525 patients, um, and public insurance, uh, was associated with decreased rates of urinary incontinence. and ARM uh patients. As always, you can find the article's linked below. We obviously need a lot more further studies to, uh, investigate this. Um, my commentary on this would be is I wonder, um, since the PCPLC is made up of specialty centers, if we missed some of the race and ethnic disparities that exist since the patients who can afford to travel to one of these centers may be, um, not captured. The patients that don't have the means to travel, I think is really important for us as a consortium. OK, so the last topic is important concepts in cloacal reconstruction surgery. Overall, this picture here shows that there are two different types of um cloacal anomalies on that high pressure distal cholostogram and the 3D cloacogram. OK, so, what about the surgical plan? I mean, what anatomic Issues do you foresee down the road? The important points to take away that have really been published in the past couple of years are that the urethral length of about 2.5 centimeters has been measured in a lot of VCUGs of normal females. And so about 1.5 centimeters is needed. If a urethra that is too short is pulled past the bladder neck, there is a risk for. And so understanding that even for a short channel can change your operative management. A short vagina, even in a very, very otherwise shorter common channel operation may require a vaginal replacement. And then finally, rotational fluoroscopy and 3D reconstructions are key to being able to make reliable measurements for anatomy. So there you have it, the PCPLC update from the 2021 pediatric surgery update. We reviewed a number of different things, including whether or not dilations are needed after PSAP, what's the best time to do the repair for either aorectal malformations or for Hirschbung disease, and we found that similar outcomes, whether we do an early or delayed PSARP for anorectal malformations or pull through for Hirschbung disease, and we talked about a few other things like bowel management strategies for patients with anorectal malformations. Hopefully you learned something here. If you like what you heard, be sure to follow us on social media, subscribe to our YouTube channel and download the Stay Current and Pediatric Surgery app. Until next time, I'm Ellen. I'm Rod. And remember, knowledge should be free.

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