Total Colonic Aganglionosis
Everything in the library about total colonic aganglionosis — built automatically from the recorded discussions that name it
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
Content of this collection
Surgical Management
5 items
Surgical Procedures for Hirschsprung Disease
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Dr. Alberto Peña presents the surgical procedures for Hirschsprung Disease. Dr. Andrea Bischoff discusses the surgical approach to total colonic aganglionosis, common complications, irrigation, urinary sodium check, ileostomy retraction, en
video128:56 · Nov 2018
Hirschsprung Disease: Surgical Procedures
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Dr. Alberto Peña presents the surgical procedures for Hirschsprung Disease. Dr. Andrea Bischoff discusses the surgical approach to total colonic aganglionosis, common complications, irrigation, urinary sodium check, ileostomy retraction, en
video128:56 · Jan 2019
Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013
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During the Pediatric Bowel Management Course in 2013,directors DrsAndrea Bischoff, Alberto Peña and Todd Ponsky discusscontroversial/hot topics surrounding the management and diagnosis of pediatric bowel conditions.In this session, the pane
video33:57 · Jan 2019
Bowel Management for Hirschsprung's Disease Patients: Pediatric Bowel...
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During the Pediatric Bowel Management Course in 2013,directors DrsAndrea Bischoff, Alberto Peña and Todd Ponsky discusscontroversial/hot topics surrounding the management and diagnosis of pediatric bowel conditions.In this session, Dr. Albe
video34:28 · Jan 2019
Bowel Management for Hirschsprung's Disease
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This segment will provide a detailed overview of bowel management in Hirschsprung's Disease by Alberto Pena.
video34:28 · Jan 2019
Evidence & Research
1 item
Adult Outcomes: Hirschsprung Disease
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Dr. Risto Rintala presents on the outcomes of Hirschsprung's disease in adults. He discusses the functional outcomes of Hirschsprung's disease, bowel function score, GIGLI score, and aganglionosis.
video20:17 · Jan 2019
Case-Based Learning
2 items
Tricks - Total Colonic Aganglionosis Associated with Malrotation & Multiple...
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Dr. Sahned Jaafar presents a difficult case of total colonic aganglionosis with malrotation and Hirschsprungs disease. The presentation is followed by a comprehensive discussion of surgical approaches to this case.
video15:41 · Nov 2018
The Colorectal Quiz Episode 10: Total Colonic Hirschsprung Disease Part 1
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We've discussed Hirschsprung Disease a lot on this podcast but we've never talked about one of the most complex forms - total colonic hirschsprung disease. Here Dr. Levitt and Dr. Frischer discuss the diagnosis and management with a case sc
podcast12:24 · Apr 2021
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Every expert statement below comes from the recorded discussions, with its speaker and moment.
Surgical Procedures for Hirschsprung Disease
Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.
clinicalAlberto Peña3:42 ↗
The concordance between pathology and radiology is 75% in rectosigmoid Hirschsprung patients, meaning 25% are discordant.
host_summaryTodd Ponsky5:30 ↗
Concordance between pathology and radiology is only 25% for long-segment Hirschsprung disease.
host_summaryTodd Ponsky5:39 ↗
If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.
opinionAlberto Peña6:28 ↗
When starting laparoscopically, you can look at the bowel and often tell what looks normal vs. abnormal, then take a biopsy at that level.
clinicalTodd Ponsky7:07 ↗
If you start transanally and break through the peritoneum, it can be difficult to maintain pneumoperitoneum when you then go laparoscopically.
clinicalTodd Ponsky7:57 ↗
Laparoscopic dissection is easy and gives you a head start when doing the transanal portion.
opinionTodd Ponsky8:18 ↗
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
clinicalAndrea Bischoff8:59 ↗
The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).
opinionAlberto Peña11:10 ↗
A bad surgeon will damage the patient both ways—laparoscopically or transanally.
opinionAlberto Peña11:37 ↗
The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.
clinicalAlberto Peña11:45 ↗
Patients complain about fecal incontinence, not the size of the scar.
opinionAlberto Peña12:17 ↗
When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.
clinicalAlberto Peña14:13 ↗
Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.
clinicalAlberto Peña14:55 ↗
The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.
clinicalAlberto Peña15:21 ↗
Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.
clinicalAlberto Peña15:48 ↗
Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.
clinicalAlberto Peña16:08 ↗
A two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.
clinicalAlberto Peña16:31 ↗
Keys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.
clinicalAlberto Peña17:12 ↗
Prone position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.
opinionAlberto Peña17:22 ↗
Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.
opinionAlberto Peña18:30 ↗
Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.
clinicalAlberto Peña18:42 ↗
The majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.
clinicalAlberto Peña21:33 ↗
A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.
clinicalAlberto Peña21:57 ↗
When you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.
clinicalAlberto Peña22:06 ↗
The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.
opinionAlberto Peña22:21 ↗
When we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.
clinicalAlberto Peña22:39 ↗
Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.
clinicalAlberto Peña22:50 ↗
After removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.
clinicalAlberto Peña23:08 ↗
Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.
clinicalAlberto Peña23:22 ↗
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