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Tricks - Total Colonic Aganglionosis Associated with Malrotation & Multiple...

Video Published 2018-11-10 Updated 2026-08-01

Timestops (5)

Case Presentation: Multiple GI Anomalies

Presenter introduces a rare case of an infant with delayed meconium passage who developed signs of Hirschsprung disease …

Clinical Questions and Literature Review

Presenter asks about optimal feeding regimen for slow weight gain, best definitive procedure (Martin vs. Duhamel vs. Soa…

Faculty Discussion: Surgical Approach and Timing

Faculty discuss the association of malrotation and Hirschsprung disease, emphasizing the teaching point that persistent …

Nutritional Management and Procedure Selection

Faculty emphasize importance of checking sodium levels in ileostomy effluent and urine to guide sodium supplementation, …

Final Comments and Closing

Brief discussion of continence concerns with low anastomosis in total colonic disease. Host thanks participants and prov…

Topic Overview

A case presentation of an infant with total colonic aganglionosis (Hirschsprung disease) associated with malrotation and multiple congenital bands—a combination the presenter reports as the first of its kind. The infant presented with delayed meconium passage and subsequently developed signs of Hirschsprung disease at day 7 of life. Surgical exploration revealed malrotation with three congenital bands and collapsed colon; biopsies confirmed aganglionosis extending to the terminal ileum. The discussion centers on optimal feeding strategies for slow weight gain, choice of definitive surgical procedure (Duhamel vs. Soave vs. Martin modification), timing of definitive repair, and continence outcomes. Faculty emphasize that no single procedure is definitively superior for long-segment disease, that sodium loss from ileostomy is a common cause of poor weight gain, and that timing of repair should be guided by stool consistency rather than age or weight alone.

Key Takeaways

Malrotation + Hirschsprung coexist rarely; if post-Ladd baby doesn't open up, consider total colonic aganglionosis. (5:42)
Delay definitive repair until ileostomy output firms up (solid food); liquid output predicts poor outcomes. (7:05)
Poor weight gain with ileostomy? Check effluent sodium (>5-7 mEq/L causes failure to thrive); supplement sodium routinely. (10:01)
No procedure (Duhamel/Soave) is superior for long-segment disease; continence outcomes ~50% regardless of technique. (7:55)
Monitor urinary sodium to guide replacement dosing; serum sodium stays normal for months despite total-body depletion. (10:01)

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

Doctor Jafar — guest
Speaker 2 — host
Speaker 3 — guest
Speaker 4 — guest
Sharif — guest
Speaker 6 — guest

Chapters

0:00Case Presentation: Multiple GI Anomalies — Presenter introduces a rare case of an infant with delayed meconium passage who developed signs of Hirschsprung disease at day 7. Surgical exploration revealed malrotation with three congenital bands, collapsed colon, and biopsies confirmed total colonic aganglionosis extending to terminal ileum. Ileostomy was created and infant placed on high-calorie formula with vitamin B12.
3:30Clinical Questions and Literature Review — Presenter asks about optimal feeding regimen for slow weight gain, best definitive procedure (Martin vs. Duhamel vs. Soave), risk of incontinence, and timing of definitive repair. Reviews literature showing only a few reported cases of total colonic aganglionosis with malrotation, and states this is the first reported case with all three anomalies (total colonic aganglionosis, malrotation, and congenital bands).
5:32Faculty Discussion: Surgical Approach and Timing — Faculty discuss the association of malrotation and Hirschsprung disease, emphasizing the teaching point that persistent obstruction after Ladd procedure should prompt consideration of Hirschsprung disease. Discussion of preferred procedures (Duhamel vs. Soave) and timing based on ileostomy output consistency rather than age or weight. Faculty note that good continence outcomes in long-segment disease occur in only about 50% of patients.
8:43Nutritional Management and Procedure Selection — Faculty emphasize importance of checking sodium levels in ileostomy effluent and urine to guide sodium supplementation, as sodium loss is a common cause of poor weight gain. Discussion of procedure choice based on surgeon experience rather than disease extent, and importance of waiting for adequate growth and stool consistency before definitive repair. Iron deficiency noted as long-term concern.
12:46Final Comments and Closing — Brief discussion of continence concerns with low anastomosis in total colonic disease. Host thanks participants and provides information about CME credit, video archive availability, and upcoming educational events.

Key claims

0:41Infant presented with delayed passage of meconium of more than 48 hours — Doctor Jafar
0:53At 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 — Doctor Jafar
1:08Full thickness rectal biopsy confirmed the absence of ganglion cells — Doctor Jafar
1:22Surgical exploration revealed malrotation with three bands: one between loops of bowel, one between bowel and liver, and one between bowel and abdominal wall — Doctor Jafar
1:56Biopsies from appendix and terminal ileum proved to be aganglionosis (Hirschsprung disease) — Doctor Jafar
2:17Infant was placed on special high-calorie formula (Ensure) with addition of vitamin B12 — Doctor Jafar
2:28Infant developed multiple attacks of dehydration requiring hospital admission for IV fluid replacement — Doctor Jafar
2:37At 70 days of age, infant's weight was 3.5 kg — Doctor Jafar
3:39Only a few cases reported in literature of total colonic aganglionosis associated with malrotation: Philone had 4 patients, Kors had 1 patient, and 3 patients reported by others — Doctor Jafar
4:00No cases reported to have all three anomalies (total colonic aganglionosis, malrotation, and congenital bands) — Doctor Jafar
4:08Congenital bands are rare and only a few cases reported; etiology unknown but could be attributed to abnormal rotation of bowel — Doctor Jafar
5:42Malrotation and Hirschsprung disease can present together; typical scenario is child with bilious vomiting who undergoes Ladd procedure but then doesn't open up, leading to discovery of total colonic aganglionosis — Speaker 3
6:06Teaching point: once Ladd procedure is done, if baby doesn't open up, must think about other potential causes for bilious vomiting including Hirschsprung disease — Speaker 3
6:18When malrotation and Hirschsprung coexist, they are usually short-segment Hirschsprung, not usually total colonic — Speaker 3
6:50For 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
7:05Timing of definitive repair should be based on consistency of ileostomy output, not age or weight; prefer to wait until output firms up, which usually happens when infant gets onto solid food — Speaker 3
7:14Infants don't do very well if definitive repair is done too early when ileostomy output is still very liquid — Speaker 3
7:36For long-segment or total colonic Hirschsprung, should leave a relatively short piece of colon, almost making a small reservoir, not the long Martin modification element — Speaker 4
7:55Good continence control in long-segment Hirschsprung disease is really about 50% of patients; the data really isn't that great — Speaker 4
8:22Long-segment Hirschsprung involving more than 50 cm from the ileocecal valve is a much more progressive disease with bigger dysmotility element — Speaker 4
8:36Cannot expect simple fix from classic operations when small bowel is significantly involved in Hirschsprung disease — Speaker 4
8:54No evidence in literature that any particular procedure (Duhamel vs. Soave) is better for long-segment Hirschsprung; best approach is to do what you do best — Sharif
9:09Duhamel may have higher episodes of enterocolitis compared to other procedures — Sharif
10:01Poor weight gain in infant with ileostomy often due to sodium loss; unless sodium levels in effluent are checked, this will not be caught because serum sodium will be normal for many months — Sharif
10:21If ileostomy output contains more than 5 to 7 milliequivalents per liter of sodium, baby will not grow or gain weight — Sharif
10:28Must get baby gaining weight and growing before performing definitive procedure — Sharif
10:34Measuring urinary sodium is the best way to guide how much sodium replacement to give — Speaker 3
10:49Every baby with an ileostomy should probably get sodium supplementation — Speaker 3
10:50Iron deficiency is a big long-term issue in these patients that often gets forgotten — Speaker 4
11:10For total colonic Hirschsprung, must wait until baby grows and ileostomy is thicker before doing definitive repair — Speaker 6
11:29After Soave or other procedure for total colonic disease, can continue with bulking agents or anti-diarrheal agents to help patients — Speaker 6
11:44Some patients who had Duhamel abroad came back with problems including enterocolitis, obstruction, and distension of the Duhamel pouch — Speaker 6
12:05Most important thing is to monitor ileostomy output before deciding to do any definitive procedure — Speaker 6
12:19Some patients with ileoanal anastomosis developed severe perianal erosion requiring protective ileostomy before further procedures — Speaker 6
12:38Better to manage total colonic Hirschsprung patients in the long term rather than rushing to definitive repair — Speaker 6
13:04Soave procedure for regular Hirschsprung disease patients (not total colonic) does not result in incontinence if procedure is followed carefully and sphincters are not damaged — Speaker 6

Cases discussed

0:29Infant with total colonic aganglionosis, malrotation, and multiple congenital bands

Points of disagreement

6:50Optimal surgical procedure for total colonic Hirschsprung disease
- Speaker 3: Prefers Duhamel because it is simple, safe, and provides a reservoir at the bottom which Soave does not
- Sharif: No evidence that any particular procedure is superior; surgeon should do what they do best and have best results with
- Speaker 6: Prefers Soave for most cases and has good results; has seen Duhamel patients from other centers with complications including enterocolitis and obstruction
7:36Length of colonic segment to preserve in Duhamel procedure
- Speaker 4: Should leave a relatively short piece of colon as a small reservoir, not the long Martin modification element
- Speaker 6: Questions how much Duhamel length is needed to avoid future problems with distension

Open questions

What is the best feeding regimen for this infant with slow weight gain despite high-calorie formula?
What is the best definitive procedure: Martin procedure, Duhamel, Soave, or ileoanal anastomosis with pouch creation?
What is the risk of incontinence with each procedure option?
What is the best timing for definitive procedure: based on weight or age?
What is the optimal length of Duhamel pouch to avoid future complications?
How much sodium supplementation is needed and how should it be monitored?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Total Colonic Aganglionosis with Malrotation and Congenital Bands: When One Diagnosis Isn't Enough

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

Presentation

A term infant failed to pass meconium for more than 48 hours 0:41. After rectal examination, meconium passed, and the infant remained hospitalized for observation. At day 7 of life, the clinical picture clarified: abdominal distension, a tight rectum, and explosive stool after digital examination 0:53. Full-thickness rectal biopsy confirmed the absence of ganglion cells 1:08.

The Decision Point

The team planned exploratory laparotomy to create a diverting colostomy. At exploration, they found not one diagnosis but three: malrotation with three distinct congenital bands — one between bowel loops, one between bowel and liver, and one between bowel and abdominal wall — alongside a collapsed colon and dilated ileum 1:22. The surgical plan changed intraoperatively. All bands were released, and an ileostomy was created in the left upper quadrant rather than the planned colostomy. Biopsies from the appendix and terminal ileum confirmed aganglionosis extending into the small bowel 1:56.

This combination — total colonic aganglionosis, malrotation, and multiple congenital bands — has not been reported 4:00. Total colonic aganglionosis with malrotation alone appears in fewer than ten published cases 3:39. The congenital bands themselves are rare, with etiology attributed to abnormal bowel rotation 4:08.

Management and Course

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 2:17. Despite this regimen, the infant developed multiple episodes of dehydration requiring hospital readmission for IV fluid replacement 2:28. At 70 days of age, the infant weighed 3.5 kg 2:37. At four months — the time of this discussion — the infant remained on ileostomy, awaiting definitive repair.

The Transferable Judgment

The discussants identified two teaching points that extend beyond this rare constellation of findings.

First, when malrotation and Hirschsprung disease coexist, the typical scenario is a child with bilious vomiting who undergoes a Ladd procedure but then fails to resume bowel function 5:42. One of the discussants noted that after a Ladd procedure, if the child doesn't resume normal bowel function, you need to think about other potential causes for the bilious vomiting 6:06, including Hirschsprung disease. When the two conditions appear together, they are usually short-segment Hirschsprung, not total colonic 6:18.

Second, poor weight gain in an infant with an ileostomy — despite adequate caloric intake and normal serum electrolytes — often reflects sodium loss in the effluent 10:01. "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" [q5]. If ileostomy output contains more than 5 to 7 milliequivalents per liter of sodium, the infant will not gain weight 10:21. Measuring urinary sodium guides replacement dosing 10:34, though some advocate routine sodium supplementation for all infants with ileostomies 10:49. Iron deficiency is another long-term issue that is often overlooked 10:50.

Timing and Technique for Definitive Repair

For total colonic Hirschsprung disease, one discussant prefers the Duhamel procedure because it is simple, safe, and provides a reservoir at the anal anastomosis, which the Soave does not 6:50. However, another noted that "the best is still what you do best" [q4] — no evidence supports one procedure over another for long-segment disease 8:54, though Duhamel may carry higher enterocolitis rates 9:09.

Timing matters more than technique. "I don't base the timing on either age or weight. I base it on the consistency of the ileostomy output" [q2]. Infants do poorly if repair is attempted while ileostomy output remains liquid 7:14. Most discussants agreed: wait until the output firms up, which typically occurs when the infant transitions to solid food 11:10. The infant must be gaining weight before definitive repair 10:28.

If a short segment of colon is left to form a small reservoir rather than a long Martin modification, outcomes may improve 7:36. Still, expectations must be realistic: "Good control in long segment Hirschprung's disease is really about 50% of the population" [q3]. When more than 50 cm of small bowel from the ileocecal valve is involved, the disease becomes more progressive, with a larger dysmotility component 8:22. The classic operations cannot be expected to provide a simple fix in these cases 8:36.

After repair, bulking agents or anti-diarrheal medications may help manage stool consistency 11:29. Some patients who underwent Duhamel developed complications including enterocolitis, obstruction, and distension of the Duhamel pouch 11:44. Severe perianal erosion after ileoanal anastomosis may require a protective ileostomy before further intervention 12:19. Long-term management is preferable to rushing definitive repair 12:38.

Takeaways from this story

After Ladd procedure for malrotation, persistent obstruction should prompt evaluation for Hirschsprung disease.
Poor weight gain with ileostomy often reflects sodium loss; check effluent sodium if >5-7 mEq/L despite normal serum levels.
Timing of repair for total colonic Hirschsprung depends on ileostomy output consistency, not age or weight.
Good continence after long-segment Hirschsprung repair occurs in only about 50% of patients.
No evidence supports one procedure over another for long-segment disease; do what you do best.

Total Colonic Aganglionosis with Malrotation and Congenital Bands in a Newborn

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

An infant presented with delayed passage of meconium beyond 48 hours 0:41. 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 — the classic triad of Hirschsprung disease 0:53. Full-thickness rectal biopsy confirmed the absence of ganglion cells 1:08.

The Decision Point

The surgical plan was straightforward: exploratory laparotomy to create a diverting colostomy. But the operative findings forced a complete reassessment. The team encountered malrotation with three separate congenital bands — one between bowel loops, one tethering bowel to liver, and one fixing bowel to the abdominal wall 1:22. The colon was collapsed. The ileum was dilated. Biopsies from the appendix and terminal ileum returned positive for aganglionosis 1:56. This was not segmental Hirschsprung disease. This was total colonic aganglionosis, malrotation, and multiple congenital bands — a combination not previously reported in the literature 4:00.

The team released all bands and created an ileostomy in the left upper quadrant. The infant was started on total parenteral nutrition, then transitioned to a high-calorie formula with vitamin B12 supplementation 2:17. But the postoperative course was difficult. The infant developed recurrent episodes of dehydration requiring hospital readmission for intravenous fluid replacement 2:28. At 70 days of age, the infant weighed 3.5 kg 2:37 — slow weight gain despite adequate caloric intake.

The question presented to the group: What is the best definitive procedure for total colonic aganglionosis, and when should it be performed?

What the Discussants Recommended

The group converged on several principles. First, the choice of operation — Duhamel versus Soave — matters less than the surgeon's experience with the technique. "The best is still what you do best," one discussant stated [q4]. There is no evidence in the literature that any particular procedure is superior for long-segment disease 8:54. One surgeon favored Duhamel for its simplicity, safety, and the small reservoir it provides at the anastomosis 6:50. Another favored Soave, noting good results in total colonic cases without incontinence when the procedure is performed carefully and the sphincters are preserved 13:04. If Duhamel is chosen, the colon should be kept short — a small reservoir, not the long Martin modification 7:36.

Second, timing should not be dictated by age or weight. It should be dictated by the consistency of the ileostomy output 7:05. "I don't base the timing on either age or weight. I base it on the consistency of the ileostomy output," one discussant explained [q2]. Infants do poorly if definitive repair is performed while the ileostomy output is still liquid 7:14. The output typically firms up when the infant transitions to solid food.

Third — and this was emphasized as the likely explanation for the poor weight gain — sodium loss through the ileostomy must be measured and replaced. Serum sodium remains normal for months even as the infant loses sodium in the effluent, and unless sodium levels in the ileostomy output are checked, the problem goes undetected 10:01. If the effluent contains more than 5 to 7 milliequivalents per liter of sodium, the infant will not gain weight 10:21. Measuring urinary sodium is the best guide for replacement 10:34, and some argued that every infant with an ileostomy should receive sodium supplementation 10:49. The infant must be gaining weight before any definitive procedure is attempted 10:28.

Finally, the group tempered expectations. Continence outcomes in long-segment Hirschsprung disease are modest — approximately 50% of patients achieve good control 7:55. When more than 50 cm of small bowel proximal to the ileocecal valve is involved, the disease becomes more progressive, with a larger dysmotility component 8:22. The classic operations cannot be expected to fully correct that 8:36.

Outcome

At the time of the discussion, the infant was four months old and awaiting definitive repair. The outcome of that repair was not reported.

What the Case Changes

This case adds a rare anatomic combination to the literature, but its teaching value lies elsewhere. First, when a child with malrotation fails to decompress after a Ladd procedure, Hirschsprung disease must be considered 6:06. Second, poor weight gain in an infant with an ileostomy is often due to sodium loss, not inadequate calories — and serum sodium will not reveal the problem until late 10:01. Check the effluent. Third, the timing of definitive repair in total colonic aganglionosis should be guided by stool consistency, not by the calendar or the scale 7:05. And fourth, long-segment disease carries a 50% chance of poor continence 7:55 — a fact that should shape the conversation with families from the outset.

Takeaways from this story

After Ladd procedure, persistent obstruction should prompt evaluation for Hirschsprung disease, not just repeat imaging.
Poor weight gain with ileostomy is often sodium loss; serum sodium stays normal for months while effluent sodium depletes the infant.
Definitive repair timing in total colonic aganglionosis should be based on stool consistency, not age or weight.
Long-segment Hirschsprung disease achieves good continence in only about 50% of patients regardless of procedure chosen.
No evidence supports one pull-through technique over another for long-segment disease; surgeon experience matters most.

Duhamel versus Soave for Total Colonic Aganglionosis: Unresolved Choices

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

# Duhamel versus Soave for Total Colonic Aganglionosis: Unresolved Choices in a Rare Disease

When the entire colon lacks ganglion cells and 50 cm or more of terminal ileum may be involved, the choice of pull-through procedure becomes consequential. The discussants in this case — an infant with total colonic aganglionosis, malrotation, and congenital bands — hold genuinely different positions on which operation to perform and when to perform it.

The Question

What is the optimal pull-through procedure for total colonic Hirschsprung disease, and does the extensive aganglionosis favour one technique over another?

The Case for Duhamel

One discussant prefers Duhamel for total colonic disease because it is simple, safe, and provides a reservoir at the bottom which Soave does not 6:50. This is a functional argument: the colonic pouch created in the Duhamel procedure may buffer the liquid effluent from a very proximal anastomosis. The same discussant bases timing not on age or weight but on the consistency of ileostomy output, preferring to wait until it firms up, which usually happens when the infant gets onto solid food 7:05. Infants don't do very well if definitive repair is done too early when ileostomy output is still very liquid 7:14.

A second discussant agrees that for long-segment or total colonic disease, the surgeon should leave a relatively short piece of colon, almost making a small reservoir, not the long Martin modification element 7:36. This is a technical refinement within the Duhamel approach — the goal is a functional pouch, not a long redundant segment that may obstruct or distend.

The Case for Surgeon Preference Over Procedure Type

Another discussant reviewed the literature and found no evidence that any particular procedure is superior for long-segment Hirschsprung; the best approach is to do what you do best 8:54. This is an argument from equipoise and surgical reality. The discussant acknowledges that Duhamel may have higher episodes of enterocolitis compared to other procedures 9:09, which offsets the theoretical reservoir advantage. The implication is that surgeon experience and institutional outcomes matter more than procedure selection in a disease where no operation reliably produces good continence.

The Case for Soave

A third discussant does not perform Duhamel, does most cases as Soave, and reports good results 11:29. This discussant has seen patients who had Duhamel abroad come back with problems including enterocolitis, obstruction, and distension of the Duhamel pouch 11:44, which raises the question of how much Duhamel length is needed to avoid future problems. The discussant agrees that for total colonic disease, the surgeon must wait until the baby grows and the ileostomy is thicker before doing definitive repair 11:10, and that after Soave or other procedure, bulking agents or anti-diarrheal agents can help patients 11:29. When challenged about continence risk with Soave, the discussant states that Soave procedure for regular Hirschsprung disease patients (not total colonic) does not result in incontinence if the procedure is followed carefully and sphincters are not damaged 13:04.

Where They Agree

All three agree on several critical points. The infant must gain weight before any definitive repair 10:28. Poor weight gain in an infant with ileostomy is often due to sodium loss; unless sodium levels in effluent are checked, this will not be caught because serum sodium will be normal for many months 10:01. If ileostomy output contains more than 5 to 7 milliequivalents per liter of sodium, the baby will not grow or gain weight 10:21. Measuring urinary sodium is the best way to guide sodium replacement 10:34, and every baby with an ileostomy should probably get sodium supplementation 10:49. Iron deficiency is a big long-term issue in these patients that often gets forgotten 10:50.

They also agree that outcomes are limited. Good continence control in long-segment Hirschsprung disease is really about 50% of patients; the data really isn't that great 7:55. Long-segment Hirschsprung involving more than 50 cm from the ileocecal valve is a much more progressive disease with a bigger dysmotility element 8:22, and one cannot expect a simple fix from classic operations when small bowel is significantly involved 8:36. Some patients who had ileoanal anastomosis developed severe perianal erosion requiring protective ileostomy before further procedures 12:19. It is better to manage total colonic Hirschsprung patients in the long term rather than rushing to definitive repair 12:38.

What Would Resolve It

The discussants did not identify a trial or dataset that would settle the question. The literature contains meta-analyses but no clear winner. The disagreement persists because total colonic aganglionosis is rare, outcomes are measured over years, and no single centre accumulates enough cases to generate definitive comparative data. Until then, the choice remains a matter of surgical judgment, institutional experience, and honest counselling about the 50% continence threshold that no procedure reliably exceeds.

Takeaways from this story

No procedure reliably produces continence above 50% in total colonic Hirschsprung; counsel families accordingly.
Check ileostomy effluent sodium; if >5-7 mEq/L, the infant will not gain weight despite adequate calories.
Defer definitive repair until ileostomy output firms up, not based on age or weight alone.
If Duhamel is chosen, leave a short colonic reservoir, not the long Martin modification element.

Keywords

Hirschsprung disease total colonic aganglionosis malrotation congenital bands delayed meconium passage abdominal distension dehydration enterocolitis sodium loss iron deficiency incontinence dysmotility full thickness rectal biopsy laparotomy ileostomy

Hashtags

#HirschsprungsDisease #Malrotation #GastrointestinalAnomalies #PediatricSurgery #Colostomy #Enterocolitis #Ileostomy #NutritionalSupport #SodiumLoss #IronDeficiency #SurgicalTechniques #PediatricNutrition #BowelObstruction #CongenitalAnomalies #TotalParenteralNutrition #SurgicalOutcomes #PediatricGastroenterology #ClinicalCase #MedicalEducation #SurgicalEducation #HirschsprungDisease #NeonatalSurgery

Transcript

Yeah, I am, I am here. Perfect. Yeah, go ahead. Would you, would you like us to advance the slides for you, or do you want to do it yourself? Uh, pardon? Would, would you like me to move the slides for you? Yeah, I would not, uh, my kids. OK, go ahead. Yeah, good afternoon, and other members of the faculty. I am going to present a very rare case of multiple GI anomaly. Next month. I put that 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 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 transverse colostomy, but on exploration we observed the following finding. We found more 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. Next slide please. During the surgery we perform that procedure, we release all bands and we create dystony just as a concealment. We take biopsy from the appendix, terminal ileum, which proved to be such a lung disease. Postly we give IV fluid antibiotic with, uh, total nutrition, and after, uh, bowel mission become positive, we start on a special formula from Ensure, which is a high calorie formula with the addition of vitamin B12. Next slide please. Post, 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 health, 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 line or bully or to perform mosis with creation of 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. Now, 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 associated with maltation. Philone has had 4 patients and uh course have 1 patient and reported 3 patients not by me but no no cases reported to have all 3 anomalies next slide please. Regarding the congenital bond which present in this case it's rare and only a few cases reported to have congenital bond is unknown could be attributed to antenna 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 straight duodenum between two forces to trade with 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 uh the left upper quadrant because uh yeah the 1 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 gotta 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 Kirschprung'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 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 because long segment that Duhamel 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, 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, do have 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 it for total economic against the officially we have, uh, already, uh, against the uni term so we will excite the junction. Uh, um, I think for the sake of time we're gonna have to, to end it unless uh anyone else has a comment to make. So, uh, Doctor Jafar, thank you for presenting this final case and I wanna thank all the presenters today and all the faculty for spending uh. The day, the, the evening, the morning, whatever time it happens to be. Again, if you would click that link button, uh, on our Globalcast web, uh, web page, not the Adobe interface, but if you go to the Globalcast and click the link, then you could go ahead and take the CME post test and get your certificate, uh, and your maintenance of certification as well. Um, this, this, uh, oh, we're gonna put the link also in Adobe. Is that what you're saying, Mark? OK. Um, this video archive will be available and searchable within 2 days where you can type in any particular thing and watch just that segment. Um, and I want to invite everyone, please again to go look at, uh, the, um, exhibit hall to see if you can sign up for that, uh, minimally invasive surgery course, uh, the pediatric minimally invasive surgery course in Vail at the end of January, Doctor Rothenberg's course. Um, also, I, I want to let everyone know that we will be having, uh, pediatric trauma, uh, 2014, that will be in February. Uh, and then, uh, and, and, and we also want to tell you about a new show we're doing advanced practice providers. So, uh, uh, PAs and, and nurse practitioners, uh, we're gonna have a full day symposium for your mid-levels to watch and participate and join in, uh, overview of pediatric surgery that's gonna be in, uh, February as well. And then, uh, we have a whole slew of others, obesity and a whole slew of others coming up. So, uh, and then then a laparoscopic hernia, of course, as well. So I wanna thank everyone for joining us today. Doctor Langer, thank you for coming all the way in from up north. Faculty, thank you for, uh, staying with us for so long and being up so late. Uh, thank you very much, and, uh, we will be, uh, seeing you uh again shortly. Thank you very much and have a great day.

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