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Total Colonic Aganglionosis
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Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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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. — Alberto Peña, Surgical Procedures for Hirschsprung Disease · 17:22
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
video2:08:56 · 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
video2:08:56 · 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
Obstructive episodes and enterocolitis are frequent in total colonic aganglionosis patients after ileal pouch–anal anastomosis but are manageable with Botox and metronidazole. Adult Outcomes: Hirschsprung Disease · 19:45
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
For total colonic Hirschsprung disease, if Soave procedure is chosen, the surgeon should wait until the baby grows and the ileostomy is thicker before performing definitive repair. Tricks - Total Colonic Aganglionosis Associated with Malrotation & Multiple... · 11:06
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
Expert statements from the recordings in this collection.
Surgical Procedures for Hirschsprung Disease
Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.
Alberto Peña3:42
The concordance between pathology and radiology is 75% in rectosigmoid Hirschsprung patients, meaning 25% are discordant.
Concordance between pathology and radiology is only 25% for long-segment Hirschsprung disease.
If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.
When starting laparoscopically, you can look at the bowel and often tell what looks normal vs. abnormal, then take a biopsy at that level.
Todd Ponsky7:07
If you start transanally and break through the peritoneum, it can be difficult to maintain pneumoperitoneum when you then go laparoscopically.
Todd Ponsky7:57
Laparoscopic dissection is easy and gives you a head start when doing the transanal portion.
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
Andrea Bischoff8:59
The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).
A bad surgeon will damage the patient both ways—laparoscopically or transanally.
The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.
Alberto Peña11:45
Patients complain about fecal incontinence, not the size of the scar.
When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.
Alberto 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.
Alberto 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.
Alberto 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.
Alberto Peña15:48
Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.
Alberto 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.
Alberto 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.
Alberto 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.
Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.
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.
Alberto 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.
Alberto Peña21:33
A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.
Alberto 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.
Alberto Peña22:06
The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.
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.
Alberto Peña22:39
Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.
Alberto 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.
Alberto 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.
Alberto Peña23:22