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Total Colonic Hirschsprung Disease with Malrotation: Difficult Cases

Video Published 2019-01-11 Updated 2026-06-10

Timestops (4)

Case Presentation: Multiple GI Anomalies

Presenter describes a neonate with delayed meconium passage who developed signs of Hirschsprung disease at day 7. Laparo…

Literature Review and Imaging

Presenter reviews literature on total colonic Hirschsprung disease with malrotation (few reported cases) and congenital …

Faculty Discussion: Surgical Approach and Timing

Faculty discuss surgical options for total colonic Hirschsprung disease. One faculty member advocates Duhamel for its re…

Nutritional Management and Continence Considerations

Faculty address poor weight gain, emphasizing sodium loss through ileostomy (>5-7 mEq/L in effluent prevents growth even…

Topic Overview

A case presentation of a neonate with total colonic Hirschsprung disease, malrotation with multiple congenital bands, and a cone segment in the terminal ileum—a combination the presenter believes is the first reported. The infant underwent laparotomy with band lysis and ileostomy creation, but experienced poor weight gain and recurrent dehydration. Faculty discussion centers on surgical approach for total colonic Hirschsprung disease (Duhamel vs. Soave), timing of definitive repair based on ileostomy output consistency rather than age or weight, and the importance of sodium supplementation and monitoring in infants with ileostomies to support growth.

Key Takeaways

Delay definitive repair until ileostomy output firms up, not based on age/weight alone—liquid output predicts poor outcomes. (6:41)
Check ileostomy effluent sodium (>5-7 mEq/L causes poor weight gain); serum sodium stays normal for months despite losses. (9:18)
For total colonic Hirschsprung, Duhamel preferred for its simplicity and built-in reservoir; continence achieved in ~50% only. (6:26)
All infants with ileostomy likely need sodium supplementation; monitor urinary sodium to guide dosing and ensure growth. (10:04)
Malrotation with Hirschsprung usually presents as bilious vomiting; if bowel doesn't open post-Ladd, consider Hirschsprung. (5:20)

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

Dr. Jafar — guest
Todd — host
Speaker 3 — guest
Speaker 4 — guest
Sharif — guest
Speaker 6 — guest

Chapters

0:02Case Presentation: Multiple GI Anomalies — Presenter describes a neonate with delayed meconium passage who developed signs of Hirschsprung disease at day 7. Laparotomy revealed malrotation with three congenital bands, collapsed colon, and a cone segment in the terminal ileum. Biopsies confirmed total colonic aganglionosis. Ileostomy was created; infant experienced poor weight gain and recurrent dehydration despite nutritional support.
3:06Literature Review and Imaging — Presenter reviews literature on total colonic Hirschsprung disease with malrotation (few reported cases) and congenital bands (rare, possibly from antenatal perforation). Presents intraoperative images showing bands between bowel loops, abdominal wall, and liver; spread duodenum; and collapsed terminal ileum and colon.
5:09Faculty Discussion: Surgical Approach and Timing — Faculty discuss surgical options for total colonic Hirschsprung disease. One faculty member advocates Duhamel for its reservoir function and bases timing on ileostomy output consistency (waiting until solid food firms output) rather than age or weight. Another emphasizes that good continence outcomes in long-segment disease are only about 50%, and that small bowel involvement beyond 50 cm from the ileocecal valve indicates more global dysmotility. A third faculty member argues no procedure is definitively superior and surgeons should use the technique they perform best.
8:24Nutritional Management and Continence Considerations — Faculty address poor weight gain, emphasizing sodium loss through ileostomy (>5-7 mEq/L in effluent prevents growth even with normal serum sodium). Urinary sodium monitoring is recommended to guide supplementation. Iron deficiency is noted as a long-term issue. Discussion of continence outcomes with Soave vs. Duhamel, with one faculty reporting good Soave results in standard cases but complications with Duhamel performed elsewhere. Presenter questions whether a more proximal anastomosis (Rehbein) might improve continence.

Key claims

0:18Infant presented with delayed passage of meconium of more than 48 hours — Dr. Jafar
0:30At day 7 of age, infant presented with signs and symptoms of Hirschsprung disease including abdominal distension, tight rectum with passage of explosive stool after removing examining finger — Dr. Jafar
0:44Full thickness rectal biopsy confirmed absence of ganglion cells — Dr. Jafar
0:58On laparotomy, malrotation with multiple bands was found: one band between loops of bowel, one between bowel and liver, and one between bowel and abdominal wall — Dr. Jafar
1:14Collapsed colon and anterior ileum with typical cone segment were observed — Dr. Jafar
1:32Biopsy from appendix and terminal ileum proved total colonic disease — Dr. Jafar
1:39Postoperatively, infant received IV fluids, antibiotics, total parenteral nutrition, and after bowel function returned, a high-calorie formula (Ensure) with vitamin B12 supplementation — Dr. Jafar
2:04Infant developed multiple attacks of dehydration requiring hospital admission for IV replacement — Dr. Jafar
2:13At 70 days of age, infant's weight was 3.5 kg — Dr. Jafar
3:16Only a few cases have been reported of total colonic Hirschsprung disease associated with malrotation (Philone had 4 patients, one other author had 1 patient, Zbra reported 3 patients) — Dr. Jafar
3:26No cases have been reported with all three anomalies (total colonic Hirschsprung, malrotation, and congenital bands) — Dr. Jafar
3:46Congenital bands are rare and only a few cases have been reported; etiology is unknown but could be attributed to antenatal perforation of the bowel — Dr. Jafar
5:20The usual scenario for malrotation with Hirschsprung disease is a child with bilious vomiting who gets a contrast study showing malrotation, undergoes Ladd procedure, but then doesn't open up, leading to discovery of total colonic Hirschsprung disease — Speaker 3
5:42After a Ladd procedure, if the baby doesn't open up, you must think about other potential causes for bilious vomiting — Speaker 3
5:54Hirschsprung disease associated with malrotation is usually short segment, not total colonic — Speaker 3
6:26For total colonic Hirschsprung disease, preferred operation is Duhamel because it is simple, safe, and provides a reservoir at the bottom, which Soave does not — Speaker 3
6:41Timing of definitive repair should be based on consistency of ileostomy output, not age or weight — Speaker 3
6:50Infants don't do well if definitive repair is done too early when ileostomy output is still very liquid; better to wait until it firms up, which usually happens when they get onto solid food — Speaker 3
7:13For long segment or total colonic Hirschsprung disease, a relatively short piece of colon should be left, creating a small reservoir rather than a long Martin modification element — Speaker 4
7:32Good continence control in long segment Hirschsprung disease is achieved in only about 50% of patients — Speaker 4
7:54When small bowel involvement extends more than 50 cm from the ileocecal valve, it represents a more progressive disease with a bigger dysmotility element — Speaker 4
8:31There is no evidence in the literature that any particular procedure is definitively better for long segment Hirschsprung disease; surgeons should use the procedure they have the best results with — Sharif
9:18Poor weight gain in an infant with ileostomy receiving adequate calories and normal blood tests is often due to sodium loss — Sharif
9:49Serum sodium will remain normal for many months before decreasing, so sodium levels in the ileostomy effluent must be checked — Sharif
9:57If ileostomy effluent contains more than 5 to 7 mEq per liter of sodium, the baby will not gain weight — Sharif
10:04The baby must be gaining weight and growing before proceeding with definitive repair — Sharif
10:10Measuring urinary sodium is the best way to guide sodium replacement dosing — Speaker 3
10:10Every baby with an ileostomy should probably receive sodium supplementation — Speaker 3
10:26Iron deficiency is a long-term issue in children with ileostomies and any repairs performed — Speaker 4
10:46For total colonic Hirschsprung disease, if doing Soave or other procedure, must wait until baby grows and ileostomy is thicker, then can continue with bulking agents or antidiarrheal agents — Speaker 6
11:20Some patients who had Duhamel performed abroad came back with enterocolitis, obstruction, and distension of the Duhamel pouch — Speaker 6
11:42Monitoring ileostomy output before deciding on any procedure is critical — Speaker 6
11:42Some patients who had ileal-anal anastomosis developed severe erosive perianal skin breakdown requiring protective ileostomy before further procedures — Speaker 6
12:40In regular (non-total colonic) Hirschsprung disease patients treated with Soave, incontinence is not seen if the procedure is performed well without damaging sphincters — Speaker 6

Cases discussed

0:02Neonate with total colonic Hirschsprung disease, malrotation with three congenital bands, and cone segment in terminal ileum

Points of disagreement

6:26Preferred surgical procedure for total colonic Hirschsprung disease
- Speaker 3: Prefers Duhamel because it is simple, safe, and provides a reservoir, unlike Soave
- Sharif: No procedure is definitively superior; surgeons should use the technique they have best results with
- Speaker 6: Prefers Soave with good results in standard cases; has seen complications with Duhamel performed elsewhere
10:10Best method to guide sodium supplementation in infants with ileostomies
- Sharif: Check sodium levels in ileostomy effluent (>5-7 mEq/L prevents growth)
- Speaker 3: Measuring urinary sodium is the best way to guide sodium replacement dosing

Open questions

What is the best feeding regimen for this infant to improve weight gain?
What is the best definitive procedure: Martin, Rehbein, Duhamel, or ileostomy with ileal pouch creation?
What is the risk of incontinence with each procedure?
What is the best timing for definitive procedure: based on weight or age?
What is the optimal length of the Duhamel pouch to avoid future complications such as enterocolitis and obstruction?

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:

Duhamel vs. Soave vs. Surgeon Preference: Operative Choice in Total Colonic Hirschsprung Disease

The points where the speakers disagreed, with each position presented side by side. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Points of disagreement · AI-written, human-reviewed

The Question

Which pull-through procedure should be performed for total colonic Hirschsprung disease — and does the extent of aganglionosis itself dictate the choice?

Position One: Duhamel for Its Reservoir Function

One discussant advocates straightforward Duhamel as the preferred operation for total colonic disease 6:26. The rationale is threefold: technical simplicity, safety, and the creation of a colonic reservoir at the anastomosis — something Soave does not provide 6:26. This reservoir function becomes relevant when the entire colon is aganglionic and the pull-through brings ileum directly to the rectum. The discussant emphasizes that modern Duhamel technique should employ a relatively short colonic segment rather than the longer Martin modification, creating a small reservoir rather than an extended pouch 7:13. This position treats the extent of disease as a factor that influences operative selection.

Position Two: Use What Works in Your Hands

Another discussant argues that no procedure has been proven definitively superior for long segment disease 8:31. After reviewing multiple meta-analyses, this surgeon concludes that the best operation is the one the individual surgeon has the best results with 8:31. While a reservoir might seem intrinsically advantageous, Duhamel carries its own risks — including potentially higher rates of enterocolitis and the mechanical complications of pouch distension and obstruction that were described in patients who underwent the procedure elsewhere 11:20. This position rejects the premise that total colonic involvement should drive technique selection and instead prioritizes surgeon experience and institutional outcomes.

Position Three: Soave Without Compromise

A third discussant performs Soave for total colonic cases and reports good results 10:46. This surgeon has observed complications in Duhamel patients referred from other centers — specifically enterocolitis, obstruction, and distension of the Duhamel pouch 11:20. The counterargument to concerns about continence is direct: in standard (non-total colonic) Hirschsprung patients treated with Soave, incontinence is not seen if the procedure is performed well without damaging the sphincters 12:40. This position holds that technical execution matters more than procedure selection, though it acknowledges that timing is critical — the ileostomy output must thicken before definitive repair, and postoperative management with bulking or antidiarrheal agents may be necessary 10:46.

Where They Agree

All three discussants converge on several points. First, timing of repair should be based on ileostomy output consistency rather than age or weight 6:41. Infants do poorly if repair is performed while output remains liquid; firming typically occurs with introduction of solid food 6:50. Second, preoperative optimization is mandatory — the infant must be gaining weight before any pull-through is attempted 10:04. Third, outcomes in long segment disease are sobering regardless of technique: good continence control is achieved in only about half of patients 7:32. When small bowel involvement extends substantially from the ileocecal valve, the disease represents a more progressive process with a larger dysmotility component 7:54, and no operation can be expected to deliver the results seen in short segment disease.

What Would Resolve It

The discussants themselves identify what is missing: high-quality comparative data. One explicitly states that despite multiple meta-analyses and reviews, no evidence in the literature demonstrates that any particular procedure should be performed specifically because the patient has long segment disease 8:31. The question is not whether Duhamel, Soave, or another technique can work — all can — but whether one consistently outperforms the others in this specific population when performed by experienced surgeons. That trial has not been done, and in its absence, the choice remains a matter of surgical judgment shaped by training, institutional experience, and individual results.

The case under discussion — total colonic Hirschsprung disease with malrotation and congenital bands — is sufficiently rare that no single center will generate comparative data 3:16. The practical resolution, then, is the one the discussants arrived at: know your outcomes, optimize the patient, time the repair correctly, and set realistic expectations with families about the likelihood of good continence control 7:32.

Takeaways from this story

No procedure is proven superior for total colonic Hirschsprung; use the technique you have best results with
Good continence control in long segment disease occurs in only about half of patients regardless of operative choice
Time definitive repair by ileostomy output consistency, not age or weight; infants do poorly if repaired while output is liquid
When small bowel involvement is substantial, expect greater dysmotility and worse outcomes

Total Colonic Hirschsprung with Malrotation: Managing the Unexpected and the Aftermath

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

When the Ladd Procedure Doesn't Solve the Problem

The infant who continues to obstruct after Ladd procedure for malrotation forces a diagnostic pivot. The usual scenario: bilious vomiting prompts contrast study showing malrotation, you perform Ladd procedure, congratulate yourself on saving the baby's life — and then the baby doesn't open up 5:20. At that point you must reconsider what else could be causing persistent obstruction 5:42. Hirschsprung disease associated with malrotation is usually short segment, not total colonic 5:54, but the association exists and must be on your differential when the expected recovery doesn't materialize.

Timing Definitive Repair by What Matters

For total colonic Hirschsprung disease, base timing of pull-through on ileostomy output consistency, not age or weight 6:41. Infants do poorly if you operate when output is still very liquid; wait until it firms up, which typically happens when they transition to solid food 6:50. This is not about hitting a weight threshold or waiting an arbitrary number of months — it's about the bowel demonstrating that it can handle the functional demand you're about to place on it.

The Hidden Cause of Poor Growth

An infant with a distal ileostomy, adequate caloric intake, and normal serum labs who fails to gain weight is losing sodium 9:18. Serum sodium will remain normal for many months before it begins to fall, so you cannot rely on routine chemistry panels 9:49. Check sodium levels in the ileostomy effluent: if it contains more than 5 to 7 mEq per liter, that baby will not grow 9:57. The baby must be gaining weight before you proceed with definitive repair 10:04. Measuring urinary sodium is the best way to guide replacement dosing 10:10, and consider routine sodium supplementation for every infant with an ileostomy 10:10. This is not a rare complication — it is common enough that you should anticipate it rather than discover it after weeks of puzzling over growth curves.

Setting Realistic Expectations for Long-Segment Disease

Good continence control in long segment Hirschsprung disease is achieved in only about 50% of patients 7:32. This is not a technical failure — it is the nature of the disease. When small bowel involvement extends more than 50 cm from the ileocecal valve, you are dealing with a more progressive, global disease with a larger dysmotility component 7:54. These patients will not respond to our classic operations the way short-segment patients do. The families need to hear this before surgery, not after.

Procedure Selection: What You Do Best

There is no evidence in the literature that any particular procedure is definitively better for long segment Hirschsprung disease 8:31. If you have good results with Duhamel, do Duhamel; if you have good results with Soave, do Soave. The theoretical advantage of a reservoir must be weighed against your own outcomes and the reality that enterocolitis, obstruction, and pouch distension can complicate any approach 11:20. For regular Hirschsprung disease patients treated with Soave, incontinence is not seen if the procedure is performed well without damaging the sphincters 12:40. Technique matters more than the name of the operation.

The Long View

Iron deficiency is a long-term issue in children with ileostomies and any repairs performed 10:26. It is not urgent at three months of age, but it will become a problem if you do not anticipate it. Some patients who undergo ileal-anal anastomosis develop severe erosive perianal skin breakdown requiring protective ileostomy before further procedures can be attempted 11:42. Monitoring ileostomy output before deciding on any procedure is critical 11:42 — not just for timing, but for understanding what the child's bowel is capable of tolerating. The operation you perform today sets the trajectory for years of management.

Takeaways from this story

After Ladd procedure, persistent obstruction requires rethinking the diagnosis—consider concurrent Hirschsprung disease.
Check ileostomy effluent sodium if infant fails to gain weight despite adequate calories; >5-7 mEq/L prevents growth.
Time pull-through for total colonic Hirschsprung by ileostomy output consistency, not age or weight—wait until it firms.
Good continence in long-segment Hirschsprung occurs in only ~50% of patients; set realistic expectations preoperatively.
No procedure is definitively superior for long-segment disease—use the operation you have best results with.

Total Colonic Hirschsprung Disease with Malrotation and Congenital Bands: A Rare Triad

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 neonate presented with delayed passage of meconium beyond 48 hours 0:18. Meconium passed after rectal examination, but the infant was kept for observation. At day 7 of life, the clinical picture clarified: abdominal distension, a tight rectum, and explosive stool after digital examination 0:30. Full-thickness rectal biopsy confirmed aganglionosis 0:44.

The Decision Point

The team planned laparotomy for transverse colostomy creation — standard management for confirmed Hirschsprung disease. At exploration, they encountered findings that changed the operation entirely. Malrotation was present, with three distinct congenital bands: one between bowel loops, one tethering bowel to liver, and one fixing bowel to the abdominal wall 0:58. The colon was collapsed, and the terminal ileum showed a characteristic cone segment 1:14. Biopsies from the appendix and terminal ileum confirmed total colonic involvement 1:32.

The combination of total colonic Hirschsprung disease, malrotation, and multiple congenital bands has not been reported 3:26. Congenital bands themselves are rare; their etiology remains unknown, though antenatal bowel perforation has been proposed 3:46. The more common scenario in malrotation with Hirschsprung disease is a child with bilious vomiting who undergoes a Ladd procedure for presumed isolated malrotation, then fails to decompress, prompting the discovery of total colonic disease 5:20. After a Ladd procedure, persistent obstruction mandates reconsideration of the differential 5:42. When the two conditions coexist, Hirschsprung disease is usually short-segment, not total colonic 5:54.

Management

The team released all bands and created an ileostomy in the left upper quadrant rather than the planned colostomy. Postoperatively, the infant received IV fluids, antibiotics, and total parenteral nutrition, transitioning to a high-calorie formula (Ensure) with vitamin B12 supplementation once bowel function returned 1:39. Despite this regimen, the infant developed recurrent dehydration requiring multiple hospital admissions for IV replacement 2:04. At 70 days of age, weight was 3.5 kg — slow growth for a child now four months old 2:13.

The Weight Gain Problem

Poor weight gain in an infant with an ileostomy receiving adequate calories and normal serum studies is often sodium loss 9:18. Serum sodium remains normal for months before declining, masking the deficit 9:49. Sodium levels in the ileostomy effluent must be measured directly. If effluent contains more than 5 to 7 mEq per liter of sodium, the infant will not gain weight 9:57. Urinary sodium measurement is the most reliable guide for sodium replacement dosing 10:10, and some advocate routine sodium supplementation for every infant with an ileostomy 10:10. The infant must be gaining weight and growing before definitive repair is attempted 10:04. Iron deficiency is another long-term concern in children with ileostomies and should not be overlooked 10:26.

Timing and Choice of Definitive Repair

For total colonic Hirschsprung disease, timing of definitive repair should be guided by the consistency of ileostomy output, not age or weight 6:41. Infants do poorly if repair is performed while output remains liquid; waiting until it firms up — typically when solid food is introduced — yields better results 6:50.

Procedure choice remains debated. One approach favors the Duhamel procedure for its simplicity, safety, and creation of a small reservoir, which the Soave does not provide 6:26. When performing Duhamel for long-segment or total colonic disease, a relatively short piece of colon should be left to create a small reservoir rather than a long Martin modification 7:13. However, there is no evidence in the literature that any particular procedure is definitively superior for long-segment Hirschsprung disease; surgeons should use the procedure with which they have the best results 8:31.

Expectations must be realistic. Good continence control in long-segment Hirschsprung disease is achieved in only about 50% of patients 7:32. When small bowel involvement extends more than 50 cm from the ileocecal valve, the disease is more progressive, with a larger dysmotility component 7:54. Some patients who underwent Duhamel developed enterocolitis, obstruction, and distension of the Duhamel pouch 11:20. Others who had ileal-anal anastomosis developed severe erosive perianal skin breakdown requiring protective ileostomy before further procedures 11:42. Monitoring ileostomy output before proceeding with any definitive procedure is critical 11:42.

What the Case Changes

This case illustrates that persistent obstruction after addressing one anatomic abnormality demands systematic reconsideration of the differential. The triad of total colonic Hirschsprung disease, malrotation, and congenital bands is exceedingly rare, but the principle is universal: when a neonate does not decompress as expected, the diagnosis is incomplete. Before definitive repair of total colonic disease, sodium balance must be addressed — serum sodium is a lagging indicator, and effluent or urinary sodium guides replacement. Finally, in long-segment Hirschsprung disease, no single operation has proven superiority; the best procedure is the one the surgeon performs best, and families must understand that even optimal surgery yields good continence in only half of patients.

Takeaways from this story

After Ladd procedure, persistent obstruction mandates reconsideration — total colonic Hirschsprung may be the hidden diagnosis.
Poor weight gain with normal serum sodium often reflects ileostomy sodium loss; measure effluent or urinary sodium to guide replacement.
Time definitive repair by ileostomy output consistency, not age or weight — wait until output firms up with solid food introduction.
No procedure is proven superior for total colonic Hirschsprung; use the operation you perform best and counsel that good continence occurs in ~50%.

Keywords

Hirschsprung disease total colonic Hirschsprung disease long segment Hirschsprung disease malrotation cone segment dehydration enterocolitis dysmotility iron deficiency sodium loss rectal biopsy full thickness rectal biopsy laparotomy ileostomy Ladd procedure

Hashtags

#HirschsprungsDisease #Malrotation #GIAnomalies #PediatricSurgery #Colostomy #Ileostomy #DuhamelProcedure #SuaveProcedure #Enterocolitis #InfantNutrition #SodiumManagement #BowelObstruction #CongenitalAnomalies #PediatricGastroenterology #NutritionalSupport #Dehydration #SurgicalBiopsy #TotalColonic #GastrointestinalSurgery #PediatricCare #HirschsprungDisease #NeonatalSurgery

Transcript

Yeah, good afternoon, Todd and other members of the faculty. I am going to present a very rare case of multiple GI anomaly. Next month. I put that on baby. Present with delayed passage of meconium of more than 48 hours, but after doing this examination, he passed meconium. Um-hum. And we keep the baby in the hospital for observation. At day 7 of his age, he presents with signs and symptoms of, uh, Hirsch disease, including abdominal distension, tight rectum with passage of explosive stool after removing the examining finger. For, uh, full thickness sector biopsy confirmed the absence of ganglion cell. Next slide please. We performed parrotomy in order to create a transverse colostomy, but on exploration we observed the following finding. We found mal rotation with multiple bands. Really it was there were 3 bands, one band between the loop of bone and the other band between the bowel and the liver. And the third one was between the bowel and abdominal wall. There were collapsed colon, anterior ileum with typical corn segment. Looks like this. During the surgery you perform that procedure, we release all bands and we create dystony just as a conciment. We take biopsy from the appendix 1al ileum, which proved to be touch a lung disease. Postparatively we give IV fluid antibiotic with a total nutrition and after, uh, bowel mission become positive, we start on a special formula from Newsure which is a high calorie formula with the addition of vitamin B12. Next slide please. Firstly, the infant developed multiple attacks of dehydration and, uh, required hospital admission of IV replacement. The infant on the day of, uh, submission of my presentation, he was 70 days, but now he is, uh, 4 months. Uh, his weight was 3.5 kg. Next slide. My consultation to Global. What is the best feeding regime for such infant because he is gaining weight slowly and what is the best definitive procedure? It is best to perform Martin procedure which prepared by some surgeon or to perform red wine or bully or to perform mosis with creation of ileal pouch. And what is the risk of incontinence? And what's the best time to perform the definitive procedure? It's related to the weight or the age? All right. Next slide. OK. Let's, let's open that up to the, uh. Yeah, regarding, uh. Go, go, go ahead, Doctor Jafar, sorry. Yeah. Regarding review of article for, uh, such similar, uh, conditions, only a few cases reported to have total chronic assciated with maltation. Philone has had 4 patients and uh have 1 patient and Zbra reported 3 patients by me, but no, no cases reported to have all 3 anomalies. Next slide please. Regarding the congenital bonds which present in this case it's rare and only a few cases reported to have congenital bond is unknown could be attributed to antenatal perforation of the bowel or finding. Uh, as far as I know, it's the first reported cases. Next slide please. This picture show bands which I removed between the loop of bowel, small bowel. The next, next slide, please. And this picture shows a band between the abdominal wall and the wrong one next slide. And this picture shows spread duodenum between two forces for trade in the absence of fixation. Next slide and uh just show configment in the terminal ileum of collapsed terminal ileum and colon until we create ostomy. As you see in this picture, we created this to me at the left upper quadrant because uh yeah the same area was in this place. All right. And thank you. Thank you very much. So, uh, tough case. Uh, so mal rotation and Hirschprung's, uh, how would you approach it? Well, I've, I've seen this a few times. Um, the usual scenario though is a child with bilious vomiting who gets an initial, uh, contrast study that shows the malrotation. So you go, you do a lab procedure and congratulate yourself. You saved the baby's life, and then the baby at that point doesn't open up and you have to go back and rethink, you know, what, what, why is the baby not opening up, and then it turns out to be total colonics. We've had a, a few kids like that, um, and that's one teaching point is that, um, once you've done a lab procedure, if they don't open up, you got to think about other potential causes for the, for the bilious vomiting. It's a great point. Um, the, uh, they're usually short segments. They're not usually total colonic. No, you're correct. Just I think it's probably in the same, the same distribution as Hirschsprung's in general, but, um, you know, we have seen a few kids with total colonic, and it's, I mean, it's the same teaching point, right? If they don't open up after the lab procedure, then you have to think, you know, what else could be causing this, uh, persistent obstruction. Um, I, I just wanted to also address the question of what operation to do for total clonic Hirschprung's and when to do it. Um, I'm sure there are lots of different opinions about that, but my, my operation is that I prefer is uh Duhamel, just a straightforward Duhamel. Um, it's simple. It's safe. Um, it does provide a bit of a reservoir at the bottom, which, uh, suave does not. Um, and I don't base the timing on either age or weight. I base it on the consistency of the ileostomy output. Um, I think they don't do very well if you do it too early when the ileostomy output is still very liquidy. I like to wait until it firms up, which usually happens when they get onto solid food. Um, and that's, that's the determining factor for me. Yeah, I, I would ask you too though, I, the Duomel is. What I've been taught and used for the long segment or total colonic, um. There was obviously having a longer segment of. Colon left and I think most of us have retreated to a relatively short piece. You're almost making a small reservoir, and I think that's uh, to me is an important piece. They no longer the long kind of element, uh, the, the, the Martin modification element. Really, it's a short piece of colon that should be there. But we should remember when you look at the data, you know, that good control in long segment Hirschprung's disease is really about 50% of the population of the people that get it done. It's the data really isn't that great. So you gotta make sure you paint the right picture to people that, that, you know, having good control going forward with the long segment. There's been a lot of discussion with the concept of long segment. Is it just, you know, total colonic or if you involve small bowel? And many people feel as you start to get, say, more than 50 or so from the, uh, ileocecal valve, it's a much more progressive disease. It makes sense. It's a more global disease. They all have a bigger dysmotility, uh, element to them. And again, you can't look for the, the simple fix of our classic operations that are gonna do the trick for them. Yeah, any comments, um, from the faculty? I was, Yeah, I, uh, I think so. Sharif, why don't you go first. Um, yeah, I, I've had a chance to look at this a few times every, every time one of these comes up, and there's been a lot of meta-analysis and reviews trying to figure out what's the best, and I think I'm convinced that the best is still what you do best. In other words, I don't think it's because long segment that Dujamel is better than a suave, although, yes, intrinsically it would sort of make sense to have a reservoir, but there also may be higher episodes of enterocolitis and other things, so. I, I don't think there's any evidence in the literature, and if there is, I'd love to see it, but I just don't think there's any evidence that any particular procedure really should be done just because the patient has long segments. So I think whatever you have had the best results with is what you should still stick with, and, and certainly my experience is very much emphasize that. So that's, in terms of that, um, that's how I would make the decision, but I, I think an important point here that we should, we should, um, Just, uh, discussed very briefly before ending is why this baby is not gaining weight, even though the, the, the, the, uh, uh, ileostomy seems quite distal, and the rest of the bowel is normal. And I think this is something that we all sometimes struggle with in a baby who's getting all the adequate calories, a blood, uh, test seem normal. And many times it has to do with sodium loss. And unless you check the sodium levels and the effluent, you will not catch that because the serum sodium will be normal for many months before it starts to decrease. But if you're getting more than 5 to 7 mL equivalents per liter of sodium out of the ileostomy, that baby will not grow. It just will not gain weight. And I think whatever procedure you're going to do, you've got to get the baby gaining weight and growing before you even get there. I think actually measuring the urinary sodium is uh is the best way to um to guide how much sodium replacement you're giving, but I mean some people say, and I think it's probably a good idea that every baby with an ileostomy should get sodium supplementation. Hmm. The other thing too is the iron deficiency in these kids though going forward is uh something that gets lost and translate not immediate at this time, you know, they're really young, but long time it's a big issue with any repairs that you end up doing that, that's forgotten. So, uh, yeah, I just a few comments um regarding uh the operation. I don't do Duhamel because I do most of my cases Suave, and I'm having a good result with that, but I think in the total colonic fishprung disease, if you decide to do this, you have to. Wait until the baby grows, until the ileostomy is a bit thicker. And then if you decide to do a wave or other procedure, you can just continue with some um uh bulking agents or um uh something like anti um Uh, diarrheal agents that can help the patients. Uh, we had a few patients that went abroad and did Duamel and actually came back with a lot of problems, enterocolitis and obstruction and distension of this Duamel. So I don't know how much Duhamel you have to do so that in the future that doesn't have any problems. Um, so anyway, um, I mean the segment or the length of the Duhamel. Uh, so I think that the most important thing is that the baby should monitor, as you said, the ileostomy output before deciding to do any procedure because we've seen patients also that had the ileoanal anastomosis and then came up with a very, very, um, uh, you know, the bottom like was very, very bad, uh, erosive, and it was um. And not not healthy, so we had to do an ileostomy to protect his anus and treat it before we do any other procedures. So I think it's better to manage these patients in the long term rather than just at this point. Very good. Uh, OK, but maybe, yeah, but maybe rebind procedure or, uh, may be better, uh, for the continence because we know it's lower, uh, anastomosis and there may be at more risk of incontinence. We do a swab for the regular Hebung disease patients, not the total colonic, and we don't see an incontinence with these patients. I think it's just you follow the the the the the the procedure very well. You should not have a damaged sphincters. I don't know. You are right, but I mean for total economic against the officially we have, uh, already, uh, again the term so we will excite the junction.

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