Colorectal Quiz: Episode 47

Published: 2025-05-08

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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

Filipe Jaldes — host
Jason Frischer — guest
Chris Geyer — guest
Mark Levitt — guest
Speaker 5 — guest

Chapters

0:00Introduction and Case Presentation — Host introduces the episode on mega-rectosigmoid and guests. Dr. Geyer presents a 13-year-old with cloaca repair at 9 months, now with daily fecal soiling.
1:34Three-System Assessment in Cloaca Patients — Discussion of urologic, gynecologic, and colorectal evaluation. Patient has normal menstruation, no urinary accidents, but daily stool accidents. Emphasis on renal function monitoring and finding a gynecology partner.
4:51Physical Exam and Prior Workup — Rectum appears well-positioned within sphincter complex without stricture. Prior cystoscopy showed narrow introitus. Anorectal manometry showed pelvic floor dysfunction but is not standard workup for ARM patients.
7:31Imaging Findings — MRI shows two hemiuteri with blood but not dilated, absent right kidney, healthy left kidney. Contrast enema reveals massive rectal dilation with normal proximal colon. Sacral ratio is 0.45.
11:31Pathophysiology and Historical Context — Discussion of why rectosigmoid dilates in ARM patients despite no stricture—both inherent motility problems and acquired from inadequate constipation treatment. Importance of preserving rectum for proprioception. Pre-1980 abdominal-perineal pull-through approach discarded rectum incorrectly.
14:47Surgical Management Options — Dr. Geyer describes rectal tapering technique with good anecdotal success. Dr. Levitt proposes Malone alone as first step, with possible later resection and sigmoid pull-through if needed. Debate over whether to preserve versus resect the dilated rectum.
20:14Final Recommendations and Case Disposition — Consensus to attempt Malone only initially, with family counseled that more definitive surgery may be needed. Acknowledgment that poor sacral ratio likely means patient will require bowel management regardless of surgical approach.
20:58Closing and Joke Segment — Patient-recorded joke and episode wrap-up with reminders to download the Stay Current app.

Key claims

1:51Every patient with cloaca history requires evaluation of three systems: urologic, gynecologic, and colorectal — Jason Frischer
3:11Spina bifida bladders and cloaca bladders need to stay empty to prevent kidney damage — Jason Frischer
3:11A 13-year-old cloaca patient historically would often show up with kidney damage, and many unfortunately ended up with renal transplant — Jason Frischer
3:11Cystatin C is helpful to check GFR and renal function in cloaca patients — Jason Frischer
3:55Female anorectal malformation patients require cesarean section for childbirth — Chris Geyer
3:55Every patient with an anorectal malformation needs a gynecology colleague to ensure they are doing well — Chris Geyer
5:09Anorectal malformation patients can have excellent anatomical repair and still have soiling — Jason Frischer
5:09The most common cause for redoing anorectal malformation patients is anus placed in the wrong position — Chris Geyer
6:29Anorectal manometry is not part of standard initial workup for anorectal malformation patients — Mark Levitt
6:29There are many ways to tell if the anus is in proper position, such as electrical stimulation and rectal ultrasound or MRI — Mark Levitt
7:14All anorectal malformation patients get an exam under anesthesia (EUA) — Jason Frischer
9:05The rectosigmoid can be very inert in ARM patients even when the anus is not strictured — Mark Levitt
10:19Mega-rectosigmoid in ARM patients can be both inherent and acquired from failure to aggressively treat constipation over many years — Mark Levitt
11:53The rectum is vitally important for bowel control in anorectal malformation patients — Jason Frischer
11:53ARM patients don't really have anal canal sensation or an internal sphincter unless the very distal aspect of the rectum was saved — Jason Frischer
11:53Distention of the rectum (proprioception) provides the cue to squeeze the external sphincter and hold in stool — Jason Frischer
11:53If you remove the rectum in ARM patients, you lose the capacity for proprioception — Jason Frischer
13:29Prior to 1980 and the PSARP, an abdominal perineal pull-through was done for ARM, throwing the rectum away and pulling sigmoid down, which was wrong — Jason Frischer
13:29Older ARM patients who had abdominal perineal pull-through can be recognized on contrast study by haustral markings at the anus and in the pelvis — Jason Frischer
14:47Removing the rectum in ARM almost guarantees incontinence — Chris Geyer
15:45Rectal tapering technique involves going in laparoscopically or open, tapering on the anti-mesenteric side with a stent or dilator in the rectum to ensure good lumen size — Chris Geyer
15:45After rectal tapering, bowel management becomes more manageable and anatomy studied one year after has not shown re-dilation — Chris Geyer
16:49Many ARM kids still have trouble with incontinence even with the best operative plans due to their anatomy and musculature — Jason Frischer
16:49A sacral ratio of 0.45 indicates the sacrum and perineal musculature are not great — Jason Frischer
17:42Colons can empty surprisingly well with antegrade enemas only, potentially avoiding resection — Jason Frischer
19:24Very often ARM patients with mega-rectosigmoid have an analplasty that is not good—either strictured, mislocated, or prolapsed — Jason Frischer
20:14If the anus is just strictured, making it bigger might allow the colon to decompress and improve — Jason Frischer

Cases discussed

1:3413-year-old female with cloaca repair at 9 months presenting with daily fecal soiling and massive rectosigmoid dilation

Points of disagreement

14:47Surgical approach to mega-rectosigmoid in this specific case
- Chris Geyer: Has performed rectal tapering both laparoscopically and open with relatively good success in similar cases, preserving the rectum
- Jason Frischer: Would remove the entire dilated rectosigmoid, pull through proximal sigmoid, and do a Malone simultaneously given the poor sacral ratio and expectation of needing bowel management anyway
- Mark Levitt: Would try Malone only first to see if antegrade enemas alone work, then consider more definitive surgery if that fails

Open questions

What was the initial state of the rectum at the time of the original cloaca repair at 9 months—was the dilation present from birth or acquired?
Will Malone procedure alone be sufficient to manage this patient's mega-rectosigmoid, or will more definitive resection be required?
Does rectal tapering maintain long-term results beyond one year of follow-up?
Can sigmoid colon provide adequate proprioception for continence if the rectum is resected, or is this expectation unrealistic?

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:

Massive Rectosigmoid Dilation After Cloaca Repair: When to Preserve the Rectum

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

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

Presentation

A 13-year-old presented with daily fecal soiling thirteen years after cloaca repair performed at nine months via perineal approach at an outside hospital 1:34. She had begun menstruating without difficulty and reported no urinary accidents, suggesting adequate gynecologic drainage and bladder control 1:34. Physical examination revealed a rectum positioned within the sphincter complex without stricture or prolapse 5:09. MRI demonstrated two hemiuteri with some blood but no dilation, an absent right kidney, and a healthy left kidney 1:34. Her sacral ratio measured 0.45 16:49. Contrast enema showed massive rectal dilation with normal proximal colon — a rectosigmoid so enlarged one discussant remarked, "this would win the Ohio State Fair" 9:05.

The Decision Point

The team faced a fundamental tension in anorectal malformation management: the rectum provides the proprioceptive signal that cues voluntary sphincter contraction 11:53. These patients lack true anal canal sensation and have minimal or no internal sphincter function unless the distal rectum was preserved 11:53. The distention of the rectum — proprioception — provides the cue to squeeze the external sphincter and hold in stool 11:53. Remove the rectum, and you eliminate that capacity 11:53.

Yet this rectum had become pathologically dilated. The discussants debated whether this mega-rectosigmoid represented inherent dysmotility or acquired dilation from years of inadequately treated constipation 10:19. One noted that the rectosigmoid can be inert in anorectal malformation patients even when the anus is not strictured 9:05. The answer, they concluded, was likely both 10:19.

Historically, before the posterior sagittal anorectoplasty revolutionized care in 1980, surgeons performed abdominal perineal pull-throughs for anorectal malformations, discarding the rectum and pulling sigmoid down — an approach now recognized as wrong 13:29. Those patients can be identified on contrast studies by haustral markings at the anus, indicating sigmoid rather than rectum in the pelvis 13:29. Removing the rectum in anorectal malformation patients almost guarantees incontinence 14:47.

Three approaches emerged: antegrade enema management alone via Malone appendicostomy, rectal tapering to preserve the organ while reducing its volume, or complete resection with sigmoid pull-through.

Management

The team initially planned a Malone procedure, acknowledging that colons can empty surprisingly well with antegrade enemas only, potentially avoiding resection 17:42. The family received explicit counseling that this might not succeed and more definitive surgery could become necessary.

Rectal tapering represented the middle path. The technique involves laparoscopic or open tapering on the anti-mesenteric side with a stent or dilator in the rectum to ensure adequate lumen size 15:45. In the limited cases where this had been performed, bowel management became more manageable and imaging one year later showed no re-dilation 15:45. The approach attempts to preserve proprioceptive function while eliminating the massive reservoir that prevents effective emptying.

Complete resection with sigmoid pull-through offered definitive treatment but sacrificed the rectum's sensory role. One discussant noted this would be appropriate only in the rare case — "a 0.1% case" — and even then would likely require concurrent Malone given the patient's poor pelvic anatomy 16:49.

The sacral ratio of 0.45 indicated that the sacrum and perineal musculature were compromised 16:49, making voluntary bowel control unlikely regardless of surgical approach. Many anorectal malformation patients still have trouble with incontinence even with optimal operative plans due to their underlying anatomy and musculature 16:49.

One critical variable remained: very often, patients with mega-rectosigmoid have an anaplasty that is inadequate — either strictured, mislocated, or prolapsed 19:24. If the anus were merely strictured, enlarging it might allow the colon to decompress and improve 20:14. If the anaplasty required revision for other reasons, the decision calculus would shift toward more aggressive resection at the time of posterior sagittal reoperation.

What This Case Reveals

The management of massive rectosigmoid dilation in anorectal malformation patients requires balancing the rectum's irreplaceable sensory function against its pathologic enlargement. The decision hinges on whether the anaplasty itself is adequate, whether the dilation appears reversible, and whether the patient's pelvic anatomy suggests any realistic chance of volitional control. When the sacrum is severely dysplastic, even perfect anatomy may not achieve continence, making bowel management programs the ultimate endpoint regardless of which colon remains.

Takeaways from this story

The rectum provides proprioceptive cues essential for sphincter control in ARM patients who lack anal canal sensation
Mega-rectosigmoid in ARM patients reflects both inherent dysmotility and acquired dilation from undertreated constipation
Rectal tapering preserves proprioception while reducing pathologic volume, with no re-dilation at one year in reported cases
Sacral ratio of 0.45 indicates poor pelvic musculature, making bowel management programs likely regardless of surgical approach
Anaplasty adequacy determines surgical strategy: stricture alone may respond to dilation, but misplacement requires revision

Managing Mega-Rectosigmoid in Anorectal Malformation: When to Preserve, Taper, or Resect

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 trainees · Explainer · AI-written, human-reviewed

Why This Subspecialty Exists

Anorectal malformations (ARM) represent a spectrum of congenital defects where the rectum fails to develop normally. When the malformation occurs in female patients with cloaca — a shared channel for urinary, genital, and intestinal tracts — the surgical complexity multiplies. These patients require lifelong multidisciplinary management because the original embryologic failure affects three organ systems simultaneously. The colorectal surgeon managing these patients must think beyond the bowel: every cloaca patient requires evaluation of urologic, gynecologic, and colorectal systems 1:51.

The Core Problem

Even after anatomically successful repair, ARM patients frequently develop massive rectal dilation — mega-rectosigmoid — that causes intractable fecal soiling. The rectum can become so inert that it fills the entire abdomen despite having no downstream obstruction 9:05. The patient presented here illustrates the dilemma: a 13-year-old with cloaca repair in infancy, normal menstruation, no urinary accidents, but daily stool accidents. Her rectum appeared well-positioned within the sphincter complex without stricture 5:09, yet contrast enema revealed massive rectal dilation.

This dilation has dual etiology. It can be inherent — motility problems present from birth — and acquired from failure to aggressively treat constipation over many years 10:19. The result is what one of the discussants called "a living creature within this child's abdomen."

Why the Rectum Matters

The central tension in managing mega-rectosigmoid is that the rectum is vitally important for bowel control in ARM patients 11:53. These patients lack anal canal sensation and typically have no internal sphincter unless the very distal rectum was preserved 11:53. Continence depends on rectal distention — proprioception — providing the cue to squeeze the external sphincter 11:53. Remove the rectum and you lose that capacity 11:53, almost guaranteeing incontinence 14:47.

This understanding represents a fundamental shift from pre-1980 practice. Before the posterior sagittal anorectoplasty (PSARP) revolutionized ARM surgery, surgeons performed abdominal-perineal pull-through borrowed from Hirschsprung's disease, discarding the rectum and pulling sigmoid down 13:29. That approach was wrong. Older patients who underwent this procedure can be identified on contrast studies by haustral markings at the anus — sigmoid colon where rectum should be 13:29.

Management Approaches

Three surgical strategies exist for mega-rectosigmoid, each with different implications:

Rectal tapering preserves the rectum while reducing its diameter. The technique involves laparoscopic or open approach, tapering on the anti-mesenteric side with a stent or dilator in the rectum to ensure adequate lumen 15:45. Anecdotal experience suggests bowel management becomes more manageable afterward, with anatomy studied one year later showing no re-dilation 15:45. This approach attempts to preserve proprioception while addressing the mechanical problem.

Resection with sigmoid pull-through removes the dilated rectosigmoid entirely and brings down proximal sigmoid. This eliminates the inert segment but sacrifices rectal proprioception. Given that removing the rectum almost guarantees incontinence 14:47, this approach essentially commits the patient to lifelong bowel management programs.

Malone antegrade continence enema (MACE) alone creates an appendicostomy for antegrade colonic washouts without resecting anything. Colons can empty surprisingly well with antegrade enemas only, potentially avoiding resection 17:42. This represents the least invasive option and can be attempted first, with more definitive surgery reserved if it fails.

When Practice Diverges

The case discussion revealed genuine disagreement about approach. One surgeon favored rectal tapering based on good anecdotal success. Another proposed Malone alone as first step, acknowledging that more definitive surgery might be needed later. A third suggested resection with sigmoid pull-through, arguing the rectum was too damaged to salvage.

The disagreement reflects uncertainty about whether mega-rectosigmoid represents reversible dysfunction or permanent structural failure. No controlled data exist to guide the choice.

The Role of Prognostic Factors

Sacral ratio — a radiographic measurement of sacral development — predicts continence potential. A ratio of 0.45, as in this case, indicates poor sacral and perineal musculature 16:49. Many ARM patients have trouble with incontinence even with optimal surgery due to their anatomy and musculature 16:49. When the sacrum is poor, the patient will likely require bowel management regardless of surgical approach, which argues for less aggressive intervention.

When to Involve This Team

Refer ARM patients with persistent soiling to a specialized colorectal center when:

Daily accidents continue despite anatomically successful repair
Contrast studies show massive rectal dilation
The anus appears malpositioned, strictured, or prolapsed — the most common cause for redo surgery 5:09
The patient reaches adolescence without achieving continence

Very often ARM patients with mega-rectosigmoid have an anaplasty that is not good — either strictured, mislocated, or prolapsed 19:24. If the anus is merely strictured, enlarging it might allow the colon to decompress and improve 20:14. But if the anus is well-positioned and the rectum remains massively dilated, subspecialty evaluation is warranted.

For cloaca patients specifically, systematic three-system evaluation is mandatory. Historically, 13-year-olds with cloaca often presented with kidney damage, and many required renal transplant 3:11. Aggressive bladder management to keep cloaca bladders empty has prevented this outcome 3:11. Check cystatin C to assess renal function 3:11, and ensure every female ARM patient has a gynecology partner 3:55 — these patients require cesarean section for childbirth 3:55.

Takeaways from this story

Rectal proprioception is the primary continence mechanism in ARM patients—removing the rectum almost guarantees incontinence.
Mega-rectosigmoid has dual etiology: inherent motility problems and acquired dilation from inadequate constipation treatment.
Malone alone may avoid resection—colons can empty surprisingly well with antegrade enemas only.
Poor sacral ratio predicts the patient will need bowel management regardless of surgical approach.
Every cloaca patient requires three-system evaluation: urologic, gynecologic, and colorectal.

Topic overview

A multidisciplinary discussion of a 13-year-old female with prior cloaca repair presenting with daily fecal soiling and massive rectosigmoid dilation despite a well-positioned, non-strictured anus. The panel debates surgical management options including antegrade continence enema (Malone) alone, rectal tapering, or complete resection with sigmoid pull-through, emphasizing the critical importance of preserving rectal proprioception for continence in anorectal malformation patients while acknowledging that her poor sacral ratio (0.45) likely precludes voluntary bowel control regardless of intervention.

Key takeaways

Rectal proprioception is critical for continence in ARM; resection almost guarantees incontinence. (11:53)
Sacral ratio 0.45 predicts poor voluntary control regardless of surgical technique chosen. (16:49)
Rectal tapering preserves proprioception while reducing dilation; anatomy stable at 1 year post-op. (15:45)
Antegrade enema (Malone) alone may decompress mega-rectosigmoid without resection. (17:42)
Always verify anus position/patency before attributing soiling to mega-rectosigmoid. (5:09)

Keywords

cloaca anorectal malformation mega-rectosigmoid fecal incontinence pelvic floor dysfunction renal agenesis spina bifida Hirschsprung's disease cloaca repair PSARP (posterior sagittal anorectoplasty) Malone procedure antegrade continence enema rectal tapering sigmoid resection abdominal-perineal pull-through

Hashtags

#ColorectalSurgery #PediatricSurgery #MedicalEducation #SurgicalQuiz

Transcript

Click "Show Transcript" to view the full text (19294 characters)

Welcome to another episode of the Colorectal Quiz. I am Filipe Jaldes, Colorectal Research Fellow at Children's National Hospital, Washington, D.C., and today we'll be discussing mega-rectosigmoid. Make sure you download the StakeCurrent app to follow along with images and other related cases. Today we are joined by Dr. Mark Levitt from Children's National Hospital and Dr. Jason Frischer from Cincinnati Children's Hospital. Welcome everyone. Glad to have you back to the Colorectal Quiz. We have some special guests today, but most importantly, I'm excited to see my good friend, Dr. Levitt. As handsome as ever, wow, I feel bad for our listeners because they don't get to see you. Yes, so they always said that I had a face for radio. Dr. Levitt will introduce our guest for this episode of the Colorectal Quiz. Welcome, Sarah Ulrich, who is coming from Yale to Cincinnati Children's to be the new Colorectal Fellow. Great to have you joining. And we have a very special guest coming all the way from Los Angeles, our good friend Chris Geyer. Welcome and congratulations, Chris, on your new physician as the Division Chief of Pediatric Surgery. And I hope that that does not take you away from your love of colorectal. And here we go with Dr. Geyer, starting us off with the case. So this patient just saw us recently, and as you mentioned, very complex. She is 13 years old. She had a cloaco repair around nine months of age at an outside hospital. And at the time of our initial evaluation, we did not have any operative reports available or any other records besides family history. As Dr. Levitt likes to remind us, every patient with a cloacoal history, we have to remember to think about three systems. We talked about this on other podcasts. You got to worry about the three systems, urologic, gynecologic, and colorectal. Okay, so this means the history needs to be focused on these systems. So be sure to investigate if they have urinary tract infections. Are they voiding well? Have they started menstruating? And are they toilet trained for stooling? And are they having any accidents? She has begun menstruating, and she's not had any issues with menstruation. And she currently endorses no urinary accidents, but does have stooling accidents daily. So it's likely that she has a good gynecologic system that at least empties her Mullerian system without pain. And she probably has a good urinary system. She's got a good bladder neck. She's voiding. She has control. She's not having urinary tract infections. We would want to check all of that. We would want to be absolutely certain that she's successfully emptying her bladder, kidney ultrasound. Cystatin C is helpful to check her GFR, her renal function. The particular attention to the renal function is a relatively new advancement in care for these children to ensure they do not require a renal transplant. I want to say that, you know, thank you to Kurt Sheldon, who was both a urologist and a pediatric surgeon by training, who was my mentor when I joined in Cincinnati in 2005 and began a wonderful friendship and partnership with Jason. He was the one that said spina bifida bladders need to stay empty. Cholaica bladders need to stay empty. And it was that move which made a huge difference in keeping those patients from having kidney damage. Because it used to be that a 13-year-old like this often would show up with kidney damage. And many of them, unfortunately, ended up with renal transplant. For redo cases, need to assess operative technique. So the history on the surgery per the family was that this operation was done entirely via a perineal approach. So it sounds to me that likely this original cholaica was relatively low because it was a perineal only repair. So in this case, it sounds like the urethra is there, the vagina is there, although the introidos made an inspection for intercourse later in life. And these patients do require cesarean section for childbirth. I can't emphasize enough how important it is to have a gynecologist that you are friendly with, that helps you, keeps you out of trouble, and sees all of the female patients with an anorectal malformation. So if you don't have one, find one, take them out to a nice dinner, order a really nice bottle of wine, and keep them close. Because every patient with an anorectal malformation needs a colleague in gynecology to make sure they're doing well. Back to our case. So in the clinic on our exam, her rectum appeared visually to be within her sphincter complex, and she had no strictures on rectal exam. So let me just add to that. Thank you for mentioning that. Is the anus okay? Yes or no? If it's perfect, they still may have soiling. That's the most incredibly frustrating thing about anorectal malformation patients. You've done an excellent anatomical repair, and they still have soiling. This patient sounds like they have a rectum that's well-located, without stricture, without prolapse. Of course, they're soiling. You've got to figure out why they're soiling. But there's no anal surgery required for this patient. But it is the first thing you need to check. And the reason why it's the first thing to check is because if the anus was placed in the wrong position, she may need surgery to redo her anal placement, which is the most common cause for redoing anorectal malformation patients. Dr. Geyer will tell us more about her workup regarding her urinary, gynecologic, and colorectal systems. So two years prior, she had come to our urologist outside of our center and had had a cystoscopy, vaginoscopy. And they commented on a narrow introitus at that time, a difficult to cannulate urethra, but they were able to ultimately cannulate it with a nine and a half French scope. And during that time, she also had anorectal mal, anorectal manometry that showed some pelvic floor dysfunction and what they called, quote, normal sensation. Dr. Frischer doesn't usually start with anorectal manometry. So we don't typically do anorectal manometry in our anorectal malformation patients as part of the standard initial workup. I never say never and I never say always, but it's not part of our standard evaluation. I think it's something that we save for circumstances that we have trouble figuring out. Because there are many ways to tell if the anus is in the proper position, such as electrical stimulation and the rectal ultrasound or MRI. That being said, often you could just tell on your physical exam and using electrical stimulation during your exam under anesthesia. You should be able to tell that by looking and by electrical stimulation. So all these patients get an EUA, which is exactly what Chris, you did. It sounds to me like the anus is now cleared anatomically. This patient is good at the perineal level, at least. So in this case, Dr. Geyer started with an MRI and be sure to check out the Stay Current app to see the pictures and follow along. Please get the, if you don't have it, the Stay Current app. And if you do have it and you're jogging right now, listening to this podcast, being inspired to run fast, you should look down on your phone because you can see a beautiful image of an MRI. And on this MRI, you can actually see- Wait, wait. But if you're driving, don't look. Wait, don't look. This is, this is from our lawyers. They just called in. They said, do not look if you're driving. Wait till you get to your, your destination. Thank you. Yes. Thank you. Thank you very much. And it's amazing that the lawyers seem to be monitoring this podcast. But the MRI shows two hemiuteri with some blood in them, but they're not at all dilated. It looks like the right tube has some fullness to it. And of course, you did mention this already, but there's obviously no kidney on the patient's right side. Good news is there's a healthy kidney on the left side. So with that, we can clear the urologic and gynecologic systems as being in working order and focus on the soiling and optimizing the colorectal system. So the next set of images, you'll see a contrast enema done two years prior that shows a rather not so subtle rectal dilation with more proximal colon being a relatively normal cow. Wow. I have one word. Wow. Jason, this would, this would win the Ohio State Fair. This is this deserves its own name. Like it's its separate organ. How you know, let's talk about this for a second. You have an anus that is not strictured. Okay. Why does this happen? It's so frustrating in ARM patients that the, the rectosigmoid can be so inert. Um, this is impressive. But Mark, this is one of those things I always wonder, cause we all have a few patients that have this anatomy like chicken or the egg. Like what came first? Cause this likely started as a quite dilated rectum from minute one. Especially you said there's a perineal repair. So you didn't, or whomever did the repair, didn't get a full view of the rectum at that time. So things do get worse over time, but what was the initial insult? Was it a motility problem or evacuation problem? Or was there some congenital distension that just perpetuated throughout childhood? So the answer is both. Um, um, it can't, it, and it can be inherent. I think the motility is inherent, the problematic that we all know that most of these patients need constipation treatment. And I also think in some respects it can be acquired from the failure to aggressively treat constipation over many, many years. It eventually dilates and dilates and dilates. So this, if you're looking at the image on your app, you see essentially a rectosigmoid, which takes up the entire abdomen pretty much. And remember, this is a patient with no stricture. I think that's key is that there's also no stricture because that could play a role, but we know that's not the case here. Of note, also very interestingly, is the transverse and left colon and the very proximal sigmoid colon looks beautiful, healthy, haustral markings, meaning there is peristalsis there. And then boom, this enormous rectosigmoid just imposes all the stasis on this poor child. Obviously this has to be managed and this brings up a very interesting treatment scenario. So let's backtrack and think about a different case. What if this was found at time of initial repair at nine months old? I want to start there and then we can talk about what you do when you're finding out. But if you went in to do your initial repair at nine months of age and had similar type of anatomy, how would you address it at that point in time? Well, I would do the usual analplasty, but then I think at the time of the colostomy closure, I might take out some of this enormity. I would leave the rectum. Generally, you want to leave the rectum because the rectum is vitally important in anorectal malformation patients. In general, you want to keep the rectum because the rectum is vital for bowel control. In an anorectal malformation patient, I cannot emphasize that importance. Why? They don't really have anal canal sensation. They don't really have an anal canal. So that's out. They don't really have any form of an internal sphincter unless the very distal aspect of the rectum was saved. Some patients have an external sphincter depending on how well formed the pelvis is, which you can determine by sacral measurements. Now, how do they know to use their external sphincter? What's the cue to squeezing the external sphincter and holding in the stool? Distention of the rectum called proprioception. So with that distention, you say, I feel something is there. I must squeeze. And then you squeeze the very nice external sphincter. If you have them, if you don't have them, then that's not a patient who's ever going to have bowel control. Okay. So that distention is key. If you remove the rectum, you lose that capacity. Dr. Levitt clue us into the prepayment world before digitalization of the PSARP approach from 1980. From a surgical point of view, Sarah, 1980 is really new for an operation. All the esophageal atresia, all that stuff was in the 50s when they started being able to get patients anesthesia and things like that. But the prior to the PSARP, what was done? The same concept that was used for Hirschsprung's disease, an abdominal perineal pull through, throw the rectum away, pull the sigmoid down. That was wrong. Obviously, the PSARP revolutionized that concept. But you may see an older patient who had an abdominal perineal pull through, and you'll recognize that because on the contrast study, you see haustral markings at the anus, in the pelvis, meaning they pulled through sigmoid because obviously the rectum has no haustral. Okay. But Dr. Levitt, what about in our case? This case is different. I want to say that. This case is different, at least in my mind. I'm not going to say exactly what I would do. But this rectum is, wow, just out of control. Like, how would one save this thing? So I'm curious to know, Chris, what you did. This is like a living creature within this child's abdomen. But I have done, in a few cases of these mega rectosigmoids in anal rectal malformations for the reasons you just so eloquently discussed of removing the rectum almost guarantees incontinence, is that I've tapered these both laparoscopically and open and at the time of colostomy closure and at the time of, after failed bowel management. And I've had relatively good success. Now the end in that is an anecdotal, you know, you could count it on definitely less than two hands, how many I've done, but it's been successful. And just a reminder, there are some images of this tapering technique on this TakeArend app. So be sure to check this out so you can picture what we are talking about here. Tell me what you actually do. I mean, literally go in there laparoscopically or open, find this large rectosigmoid, and on the anti-mesenteric side, taper. Make sure you have someone, you know, full body prep or split leg table, depending on the size and age of the child, having a stent or a dilator in the rectum. So I know that I have a good size lumen. Like a sleeve gastrectomy. Like a sleeve gastrectomy or like a proximal jejunal atresia. And I staple and I over-sow. Sometimes I over-sow, sometimes I don't. But, and I've studied these kids afterwards. First of all, their bowel management becomes more manageable. And then two, I've studied their, their, um, anatomy afterwards. And I haven't seen dilation. I've only studied anatomy like a year after. I haven't watched over many, many years, but I'm still following those patients. In many of these kids, even with the best operative plans, they still have trouble in incontinence. And frequently it's just a matter of their anatomy and their musculature. I see that the sacrum ratio is 0.45. The sacrum is not great. Um, so the perineum, the musculature is probably not great. So I think this patient is going to be a bowel management candidate. And I doubt they will ever achieve successful bowel control anyway, given the quality of their pelvis, the amount of quadruple regression that they have. So what I would do in this case, in this unique case is I would remove that entire thing and pull through the proximal sigmoid and make a beautiful, well-sized analplasty and do a Malone at the same time and say, that's the way they're going to be. And Dr. Levitt has had some patients with good success with continence where the sigmoid is able to feel some distension, but it certainly isn't the expectation. I would do a redo for this patient. I would do a redo PSARP, remove all the perineal rectum, dissect it out, go into the abdomen, dissect out this monstrosity and pull through the proximal sigmoid, close the posterior sagittal incision and having, would have done a Malone. That's a 0.1% case. The other 99 plus percent cases, I would do a sigmoid resection if necessary, but usually you don't have to do that. Usually a Malone only is all you need. And honestly, for this case, I might even consider trying that knowing that it might not work, but you'd be surprised how well colons empty with antegrade only and completely avoid a resection. In Dr. Guy, your patient, she had the initial enemas, eventually became a burden on their quality of life and the family was lost to follow up. And when she represented, she had this difficulty of incontinence and distension of the rectum. And so the question is, what is he going to do next? So we haven't done anything yet. I had planned on doing a Malone only and trying and seeing if that would work. I had not thought about the tapering idea. It does make sense. I can't imagine it'll be super easy on this, but it might be worth a try over redoing the entire thing. But yeah, I think, I think I agree with you, particularly the response to enemas was impressive. I would do Malone only, see how it goes, but I would tell the family that might not succeed and you may need to be, do something more definitive and remove some colon. And then you have the tapering option or the take the whole thing out and bring the sigmoid down option. I will say that very often you have a patient like this and the analplasty is not good. It's either strictured, mislocated or prolapsed. So you have to redo the analplasty anyway. And then I think you're faced with a very difficult decision because you have to do a PSARP of some sort, a redo PSARP of some sort. In that case, I would do the whole shebang that I just said. However, you divert the patient at that time as well? I would not. I would not divert. However, if if it was just a stricture, I might make the anus bigger and give that a chance to decompress because that colon might get a heck of a lot better. But if it was not strictured and it was this enormous, then I would say that thing Frischer said about the inherent problem. It was probably there since birth. It's probably true. I'd probably go ahead and resect it. This is a great case, Mark. Really, I love it. This is great, Chris. Thank you so much for sharing. Let us know what happens. For sure, I will. And now for our episodic colorectal quiz joke. We had a patient today who always tells me colorectal jokes. So I asked him and his parents, would he like to record his joke? And I will play it for our international audience directly with his voice. Which we've never done before. And I'm going to play it now. This is a first. Drum roll, please. All right. Here we go. Just a colorectal surgeon have in common with the astronaut. They both see Uranus. Nice. Nice. And for those of you who didn't catch that, our wonderful patient said, what does a colorectal surgeon and an astronaut have in common? And he said the answer was, they both see your anus. Phenomenal. Phenomenal. Thank you all for joining us for this episode of the colorectal quiz. And remember to download the Seekerin app from the Apple App Store or the Google Play Store to check out the images and algorithms we discussed in this episode. Additionally, remember to follow us on social media and check out our YouTube channel for more pediatric surgery content. and I'm so sure o'erh, I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can go. I can. I can. I can. I can. I can. I can. I can.

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