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Total colonic Hirschsprung disease: Ileostomy take down and ileoanal pull-through

Video Published 2026-03-19 Updated 2026-08-01

Timestops (4)

Patient History and Surgical Approach

Perineal Dissection and Rectal Mobilization

Ileostomy Takedown and Colon Removal

Ileal Pull-Through and Anastomosis

Topic Overview

Surgical management of total colonic Hirschsprung disease in a patient who presented with neonatal obstruction. After initial ileostomy and colonic mapping confirmed aganglionosis throughout the colon with ganglion cells present in the ileum, the patient underwent ileostomy takedown and ileoanal pull-through at age 1. The procedure was performed using a transanal prone rectal dissection followed by supine abdominal approach, preserving the anal canal and dentate line while creating a tension-free ileoanal anastomosis.

Key Takeaways

Prone transanal dissection clarifies anatomy even in virgin rectums, aiding future reoperations. (1:15)
Preserve dentate line by marking 0.5cm from anal verge; hide it under retraction during dissection. (1:39)
Route pull-through segment down right pelvis; confirm reach by marking 4cm below pubic symphysis. (6:31)
Martini glass vascular technique preserves V-shaped arcade while ligating stem for tension-free anastomosis. (7:08)
Two-layer anastomosis: seromuscular stitches to sphincters, then mucosa-to-mucosa at preserved anal canal. (7:59)

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

Speaker 1

Chapters

0:01Case presentation and initial management — Patient with total colonic Hirschsprung disease presented with neonatal obstruction, underwent ileostomy and colonic mapping showing aganglionosis throughout colon with ganglion cells at ileal stoma site.
0:54Transanal prone rectal dissection — Prone position transanal dissection preserving dentate line and anal canal, dissecting in full-thickness Swenson plane to distal rectum.
4:57Ileostomy takedown and colon removal — Patient repositioned supine, both ileostomy limbs taken down with elliptical incision, aganglionic colon removed, small bowel oriented for pull-through.
6:51Ileoanal pull-through and anastomosis — Ileal segment prepared preserving vascular arcade, pulled through to perineum without tension, anastomosed to anal canal with seromuscular and mucosal layers.

Key claims

0:15Patient presented with neonatal obstruction — Speaker 1
0:15Contrast enema was typical of total colonic Hirschsprung disease — Speaker 1
0:27All rectal and colonic biopsies showed no ganglion cells — Speaker 1
0:33Good ganglion cells were present at the ileum where the stoma was opened — Speaker 1
0:38Patient thrived and needed no enteral or parenteral nutrition supplementation — Speaker 1
0:46At age 1, stool was noted to be thick in the ileostomy, prompting pull-through — Speaker 1
0:57The distal ileostomy limb had about 25 centimeters of ganglionic ileum — Speaker 1
1:15All redo Hirschsprung cases are best handled prone — Speaker 1
1:21Transanal prone rectal dissection in untouched rectum helps understand anatomy of previously operated rectums — Speaker 1
1:39Dentate line should be preserved and hidden under retraction pins — Speaker 1
1:53Marking 0.5 centimeters from anal verge preserves the anal canal — Speaker 1
2:39There is a typical areolar plane in the full-thickness Swenson dissection — Speaker 1
4:40Tying off the distal rectum prevents spillage when entering the abdomen — Speaker 1
5:18Elliptical incision around stomas makes transverse incision easier to close — Speaker 1
5:32Downstream small bowel is very small in caliber after prolonged diversion — Speaker 1
6:31Best orientation for pull-through is with small bowel limb coming down the right pelvis — Speaker 1
6:37Marking 4 centimeters below superior aspect of pubic bone confirms pull-through segment will comfortably reach perineum — Speaker 1
7:02Intact vascular arcade supplies the distal ileal segment — Speaker 1
7:08Martini glass technique preserves V-shaped vessels and ligates the stem — Speaker 1
7:51Pull-through segment must be under no tension — Speaker 1
7:59Anastomosis uses seromuscular stitches from bowel to sphincters in 4 positions — Speaker 1
8:10Second anastomotic layer is mucosa of ileum to mucosa proximal to preserved anal canal — Speaker 1

Cases discussed

0:10Infant with total colonic Hirschsprung disease managed with staged approach: initial ileostomy followed by ileoanal pull-through at age 1

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 Hirschsprung: technical sequence for ileoanal pull-through

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

Total colonic Hirschsprung disease requires ileoanal pull-through after the entire colon is confirmed aganglionic. The technical decisions that determine outcome cluster around three phases: transanal dissection, abdominal mobilization, and anastomosis under no tension. Each phase contains judgment calls that separate a functional result from a struggling one.

Build pattern recognition in the untouched rectum

Perform the transanal dissection prone, even in primary cases. All redo Hirschsprung cases are best handled prone 1:15, and any opportunity to dissect an untouched rectum in this position builds the spatial understanding you will need when anatomy is distorted by prior surgery 1:21. The prone transanal approach in a primary case is practice for the hardest cases you will face.

Preserve the dentate line by hiding it under retraction pins. Place the pins to show and protect the dentate line, then mark the incision 0.5 centimeters from the anal verge 1:53. This preserves the anal canal 1:53. The dentate line should remain hidden under the pins throughout the dissection 1:39—if you can see it easily, your pins are misplaced.

Recognize the areolar plane of the full-thickness Swenson dissection. There is a typical areolar plane one can see 2:39. This is not a plane you create by force; it is a plane you find by retracting the anal canal and working in the correct tissue layer. If you are not seeing this characteristic areolar quality, you are in the wrong plane.

Anticipate the consequences of prolonged diversion

Expect the downstream bowel to be small and plan your incisions accordingly. The distal small bowel is very small in caliber after prolonged diversion 5:32. An elliptical incision around both stoma sites makes the transverse incision easier to close at the end of the operation 5:18. This is not cosmetic—it is about managing tension in a closure where one limb of bowel has been functioning and the other has been idle.

Confirm reach before committing to the pull-through segment. Mark a point 4 centimeters below the superior aspect of the pubic bone; this confirms the pull-through segment will comfortably reach the perineum 6:37. Do this before dividing mesentery. The downstream ileum in this case had about 25 centimeters of ganglionic bowel 0:57, but caliber and mesenteric mobility matter more than length on a ruler.

Preserve blood supply and eliminate tension

Use the martini glass technique to preserve the vascular arcade. The intact arcade supplies the distal ileal segment 7:02. "You want to save the V part of the glass and ligate the stem" [q5]—preserve the V-shaped vessels and ligate only the stem 7:08. This is not about saving every vessel; it is about preserving the arcade geometry that keeps the distal segment perfused.

Run the bowel to confirm orientation, then reconfirm after pull-through. The best orientation is with the small bowel limb coming down the right pelvis 6:31. Run the bowel before pulling it through to ensure proper orientation, then reconfirm after the segment is pulled through to be certain there is no twist 7:51. A twisted pull-through under tension will fail; a straight pull-through under no tension will function 7:51.

Construct a two-layer anastomosis

Anchor the bowel to the sphincters before creating the mucosal anastomosis. Place seromuscular stitches from the bowel to the sphincters in four positions 7:59. This first layer stabilizes the pull-through segment. The second layer is mucosa of the ileum to mucosa just proximal to the preserved anal canal 8:10. The seromuscular layer carries the mechanical load; the mucosal layer seals.

The operation succeeds when the pull-through segment is under no tension, properly oriented, and well-perfused. Every technical step serves one of those three goals. The patient in this case thrived after the initial diversion and needed no enteral or parenteral nutrition supplementation 0:38, which made the pull-through straightforward—but the technical principles apply even when the clinical course has been complicated.

Takeaways from this story

Perform primary transanal dissections prone to build pattern recognition for redo cases, which are always best handled prone.
Mark 4 cm below the pubic bone before dividing mesentery to confirm the pull-through segment will reach without tension.
Martini glass technique preserves V-shaped arcade vessels while ligating the stem, maintaining perfusion to the pull-through segment.
Downstream bowel is very small after prolonged diversion; elliptical stoma incisions ease closure of the transverse incision.
Run bowel before and after pull-through to confirm orientation with no twist; the segment must be under no tension.

Total colonic Hirschsprung: Technical sequence for ileoanal pull-through after diversion

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 specialists · Teaching arc · AI-written, human-reviewed

Prone dissection as deliberate training

The decision to perform the initial transanal dissection prone in a primary case is not about necessity—it is about building pattern recognition for the inevitable redo 1:15. Every untouched rectum dissected prone teaches the anatomy you will need when returning to a scarred pelvis 1:21. The areolar plane in a full-thickness Swenson dissection has a characteristic appearance 2:39, and learning to recognize it in favorable conditions means you will find it again when the tissue planes are obliterated by prior surgery.

Preserving what cannot be reconstructed

The dentate line must be hidden under the retraction pins, not exposed or violated 1:39. Marking 0.5 centimeters from the anal verge defines the preserved anal canal 1:53—this is not an approximation to adjust based on operative feel. The distance is small enough that imprecision matters. If you mark too far from the verge, you have removed functional anoderm. If you mark too close, you have left transitional mucosa that will not hold sutures reliably.

Stoma takedown geometry

The elliptical incision around both stoma sites is not cosmetic 5:18. A circular excision leaves you closing a transverse laparotomy through tissue that has been radially stretched by the stoma appliance. The ellipse converts that into a line of closure under even tension. This is the sort of detail that separates a clean closure from one that develops a hernia at eighteen months.

The downstream limb will be startlingly small 5:32. After a year of diversion, the distal 25 centimeters of ileum that saw no enteral flow is collapsed and pale 0:57. It does not predict function—this patient thrived without supplementation 0:38—but it does predict that your anastomosis will be to a narrow lumen, not the wider proximal bowel you are accustomed to.

Orientation and reach

Running the small bowel to confirm orientation is not optional 6:31. The mesentery will allow the ileum to reach the pelvis in multiple configurations, only one of which is correct. The optimal path brings the limb down the right side of the pelvis 6:31. Marking a point 4 centimeters below the superior edge of the pubic symphysis and confirming the bowel reaches it comfortably tells you the pull-through will succeed before you commit to the anastomosis 6:37. If it does not reach without tension at that mark, it will not reach the perineum without tension, and a tension anastomosis in an infant ileoanal pull-through will stricture.

Vascular preservation at the mesenteric division

The martini glass technique is named for what you preserve, not what you divide 7:08. The V-shaped arcade supplies the distal segment; the stem does not 7:02. Ligating the stem and preserving the V maintains perfusion to the entire length of bowel you need 7:08. Dividing one of the V limbs because it looks redundant leaves you with marginal perfusion to the distal portion of your pull-through, which is the portion forming your anastomosis.

The two-layer anastomosis

The first layer is seromuscular, bowel to sphincter, placed at four quadrants 7:59. This layer carries no mucosal apposition—it exists to anchor the ileum in the pelvis and prevent retraction. The second layer is mucosal, ileum to the mucosa just proximal to the preserved anal canal 8:10. If you place the mucosal layer to the anal verge instead of proximal to it, you have anastomosed to non-mucosal tissue and created a raw surface that will granulate rather than epithelialize.

The pull-through must be confirmed under no tension before placing any sutures 7:51. Tension is not something you accommodate with technique—it is something that makes the operation fail.

Takeaways from this story

Prone transanal dissection in primary cases builds pattern recognition essential for redo Hirschsprung operations.
Mark 0.5 cm from anal verge to preserve anal canal—precision here prevents removing functional anoderm or leaving unsuitable mucosa.
Elliptical stoma excision converts radially stretched tissue into a line of even tension, reducing hernia risk at closure.
Confirm pull-through reaches 4 cm below pubic symphysis without tension before committing to anastomosis—inadequate reach predicts stricture.
Martini glass mesenteric division preserves V-shaped arcade supplying distal ileum; dividing a V limb compromises perfusion to anastomosis.

Ileoanal Pull-Through for Total Colonic Hirschsprung Disease After Prolonged Diversion

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 and Initial Management

An infant presented with neonatal obstruction 0:15. Contrast enema showed findings typical of total colonic Hirschsprung disease 0:15, and colonic mapping confirmed the diagnosis: all rectal and colonic biopsies showed absent ganglion cells 0:27, with normal ganglion cells identified at the ileum 0:33. The team created a double-barrel ileostomy, leaving the functional limb with the majority of the ileum and the distal limb with approximately 25 centimeters of ganglionic ileum 0:57. The child thrived without requiring enteral or parenteral nutrition supplementation 0:38.

The Decision Point

At age one, the ileostomy output changed — stool became thick 0:46. This observation prompted the question of timing for definitive repair. In total colonic Hirschsprung disease, the decision to proceed with pull-through balances the child's nutritional status, stool consistency suggesting adequate absorptive capacity, and the technical considerations of working with small-caliber diverted bowel. The team chose to proceed with ileoanal pull-through.

The approach itself required a second decision: how to handle the rectal dissection in a patient who would eventually need revision surgery. Rather than defaulting to a supine transanal approach, the discussant elected to begin prone 1:15. The reasoning was explicit: all redo Hirschsprung cases are best managed prone, and performing the initial dissection in this position on an unoperated rectum would build the anatomic understanding needed for future revisions 1:21.

Operative Technique

The transanal dissection began with careful preservation of the dentate line, hidden under retraction pins 1:39. Marking 0.5 centimeters from the anal verge preserved the anal canal 1:53. The full-thickness Swenson dissection proceeded in a characteristic areolar plane 2:39. After tying off the distal rectum to prevent spillage when entering the abdomen 4:40, the patient was repositioned supine.

The ileostomy takedown used an elliptical incision around both stomas to facilitate transverse closure 5:18. The downstream small bowel was markedly small in caliber after prolonged diversion 5:32 — a finding that affects both the technical ease of anastomosis and expectations for postoperative function. The small bowel was run carefully to confirm proper orientation, with the optimal configuration bringing the limb down the right pelvis 6:31.

Before committing to the pull-through length, the team marked a point 4 centimeters below the superior aspect of the pubic bone, confirming the segment would reach the perineum without tension 6:37. The vascular supply to the distal ileal segment remained intact through its arcade 7:02. Using what the discussant described as a "martini glass technique" — preserving the V-shaped vessels while ligating the stem 7:08 — the ileal segment was mobilized with excellent perfusion and no tension 7:51.

The anastomosis was constructed in two layers: first, seromuscular stitches from bowel to sphincters in four quadrants 7:59, then mucosal apposition of ileum to the mucosa just proximal to the preserved anal canal 8:10.

Outcome

The discussant did not describe the postoperative course or long-term functional outcome.

What This Case Changes

The technical choices here reflect a longer view than the immediate operation. Choosing prone position for the initial rectal dissection was not about optimizing this particular pull-through — it was about building muscle memory for the inevitable revisions that total colonic Hirschsprung patients require. The careful preservation of anal canal anatomy, the attention to tension-free pull-through despite working with small-caliber diverted bowel, and the explicit acknowledgment that downstream ileum will be diminished after prolonged diversion — these are the judgments that separate adequate technique from durable results. The case demonstrates that in complex congenital surgery, the first operation should be planned with the second one already in mind.

Takeaways from this story

Prone positioning for initial rectal dissection builds anatomic understanding needed for future Hirschsprung revisions
Downstream ileum after prolonged diversion is markedly small in caliber, affecting both technical approach and expectations
Tension-free pull-through confirmed by marking 4cm below pubic bone before committing to segment length
Martini glass vascular technique preserves V-shaped vessels while ligating stem to maintain ileal segment perfusion

Keywords

total colonic Hirschsprung disease neonatal obstruction aganglionosis ileostomy colonic mapping ileostomy takedown ileoanal pull-through transanal prone rectal dissection Swenson dissection contrast enema rectal biopsy colonic biopsy

Hashtags

#HirschsprungDisease #PediatricSurgery #ColorectalSurgery #PullThrough #IleoanalPullThrough

Transcript

Total colonic Hirschrung's disease, ileostomy takedown, and ileoanal pull through. We are going to show you a case of a patient with total colonic Hirschprung's. The patient presented with neonatal obstruction, and a contrast enema was typical of total colonic Hirschprungs, and they underwent an ileostomy and colonic mapping. All biopsies, rectal and colonic, showed no ganglion cells. There were good ganglion cells at the ileum where the stoma was opened. The patient did well, thrived, and needed no enteral or parenteral nutrition supplementation. At the age of 1, the stool was noted to be thick in the ileostomy, so a pull through was offered. Here you see the double barrel ileostomy. The functional side is the majority of the ileum, and the distal side has about 25 centimeters of a ganglionic ileum. After a total body preparation, we begin the operation in prone position. I like to do a transanal prone rectal dissection in such a case. Because I know that all redo Hirschprung's cases are best handled prone. So any opportunity that I have to do a dissection with this approach in prone position in an untouched rectum, I take as it helps understand the anatomy of a previously operated on rectum dissected out in the same way. I place the lone star pins carefully to show and to preserve the dentate line. You see how we have hidden the dentate line under the pins. I now mark 0.5 centimeters from the anal verge, which preserves the anal canal. And I cut this circle. And then place sutures full thickness in the rectal wall. We now look for the full thickness Swenson plane. There is a typical areolar plane one can see. It is helpful at this stage to move the pins again and fully retract back the anal canal. The mixit or clamp helps facilitate this dissection. And now I tie off the distal rectum so there won't be any spillage when we enter the abdomen and also tie off the silk sutures. And we turn the patient supine. I take down both limbs of the ileostomy. I like to make an elliptical incision around the stomas, which makes this transverse incision easier to close at the end of the operation. We're stapling off the ends of both limbs of small bowel. You can see the downstream small bowel, which is very small in caliber. And now I'm removing the a ganglionic colon. I run the small bowel to be sure it is properly oriented as we set up for the pull-through. The best orientation seems to be with the small bowel limb coming down the right pelvis. I now mark a spot 4 centimeters below the superior aspect of the pubic bone. This location confirms to me that the pull-through segment will comfortably reach the perineum. And now we are setting up for the illuanal pull through. You can see the intact arcade which will supply the distal ileal segment. And I now draw a martini glass. You want to save the V part of the glass and ligate the stem. And the planned ileal segment is now straight and has excellent blood supply. And here we have pulled through the ileum and reconfirmed its orientation to be sure I haven't twisted it. The pull-through segment is under no tension. I place a serum muscular stitch from the bowel to the sphincters in 4 positions. And then the next layer is mucosa of the ileum to mucosa just proximal to the preserved anal canal. I moved the pins back to show the anal canal. And complete the ileoanal anastomosis.

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