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Hirschsprung Disease

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NPO babies diagnosed with Hirschsprung disease are typically managed with irrigations, antibiotics if they show evidence of enterocolitis, and NPO or NG tubes if they're distended. — Aaron Garrison, Hirschsprung Disease in Brief · 6:37

Hirschsprung's Disease
Dr. Rae Hanke, Dr. Alex Casar, Dr. Jason Frischer, and Dr. Aaron Garrison come together to provide you the essentials on diagnosis and management of Hirschprung's Disease. Intro and outro tracks are adapted from "I dunno" by grapes, featuri
podcast20:48 · Dec 2020
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Hirschsprung Disease in Brief
The surgical management for Hirschsprung disease has changed dramatically over the past few decades. So here, we discuss the basics of workup, diagnosis, and surgical treatment for aganglionic megacolon AKA Hirschsprung disease with Dr. Aar
video10:44 · Feb 2022
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Colorectal Quiz Episode 3: Hirschsprung Disease
In the third episode of the Colorectal Quiz series, Dr. Levitt and Dr. Frischer discuss the basics of the initial management and diagnosis of Hirschprung disease.2 view abdominal XRContrast enema
podcast20:10 · Feb 2021
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In settings without pathology support, empiric diversion in the dilated segment is a reasonable strategy; if that bowel works, that is where the pull-through will go. The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique · 7:07

Hirschsprung Disease Part I with Marc Levitt
Dr. Marc Levitt and Dr. Todd Ponsky discuss Hirschsprung disease.Dr. Ponsky: Welcome to "Stay Current” in pediatric surgery, I’m Todd Ponsky, a pediatric surgeon at Akron Children’s Hospital and today we’re going to be focusing on Hirschspr
podcast59:20 · Dec 2020
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Hirschsprung Disease Part II with Dr. Marc Levitt
Continuation of discussion on Hirschsprung disease with Dr. Marc Levitt with emphasis on complex situations.
podcast44:38 · Dec 2020
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The Colorectal Quiz Episode 3.5: Proximal Hirschsprung Disease
In this episode, Dr. Levitt and Dr. Frischer discuss the workup and management for a patient with suspected proximal Hirschsprung disease. Listen as they walk you through a their thought process and what to consider in these complex patient
podcast8:42 · Feb 2021
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The Colorectal Quiz Episode 4: Classic Hirschsprung disease - Surgical Technique
In this episode of the Colorectal Quiz, Dr. Marc Levitt and Dr. Jason Frischer discuss considerations when operating on a patient with classic distal sigmoid Hirschsprung disease with special guests Dr. Aaron Garrison and Dr. Andrea Badillo
podcast19:28 · Feb 2021
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The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique
In this episode of the colorectal quiz, Dr. Marc Levitt and Dr. Jason Frischer discuss the surgical considerations when a patient with Hirschsprung disease presents with a more proximal transition zone in the transverse colon or hepatic fle
podcast14:37 · Mar 2021
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The Colorectal Quiz Episode 10: Total Colonic Hirschsprung Disease Part 1
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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Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1
We've discussed Hirschsprung Disease at length before but today we visit a complicated topic that many deal with, obstruction. Dr. Levitt and Dr. Frischer speak with Dr. Rentea from Children's Mercy at Kansas City about the obstructed patie
podcast24:11 · Aug 2021
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The Colorectal Quiz Episode 20: Hirschsprung Disease Obstruction Part 2
We've discussed Hirschsprung Disease at length before but today we visit a complicated topic that many deal with, obstruction. Dr. Levitt and Dr. Frischer speak with Dr. Rentea from Children's Mercy at Kansas City about the obstructed patie
podcast21:29 · Sep 2021
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The Colorectal Quiz Episode 22: Hirschsprung Disease - the Soiling Patient Part 1
In the last few episodes of the Colorectal Quiz, we discussed obstructions in Hirschsprung disease. Now we're turning to another issue in Hirschsprung disease - the soiling patient. Along with Dr. Levitt and Dr. Frischer, we're joined by Dr
podcast19:45 · Nov 2021
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The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient Part 2 -The Dentate Line and Motility
In this episode we dive deeper into discussion regarding a patient with Hirschsprung disease who has issues with fecal incontinence. We specifically will discuss the importance of the dentate line and how motility plays a part in management
podcast26:46 · Dec 2021
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Colorectal Quiz Episode 27: Delayed Hirschsprung Disease
podcast21:40 · Feb 2022Private

In newborns with suspected Hirschsprung disease, the initial workup includes a water-soluble contrast enema looking for a narrower distal segment (rectum) with dilation above. — Nelson Rosen, Hirschsprung Disease Workup · 3:30

Hirschsprung Disease Workup
The workup for Hirschsprung disease is bread-and-butter pediatric surgery but it can certainly get confusing. Dr. Nelson Rosen is the Associate Director of the Colorectal Center at the Cincinnati Children's Hospital Medical Center and here
podcast10:44 · Aug 2021
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StatPearls: Hirschsprung Disease ↗

Sharp dissection is preferred to mobilize the submucosal space, although blunt dissection can also be used. Technique: Laparoscopic Assisted Pull-through for Hirschsprung's Disease · 7:05

Technique: Laparoscopic Assisted Pull-through for Hirschsprung's Disease
Laparoscopic Assisted Pull-through for Hirschsprung's Disease using a Just Right sealer by Dr. Steven Rothenberg.
video10:47 · Nov 2018
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Different Rectal Biopsy Techniques for Hirschsprung Disease
Abstract The diagnosis of Hirschsprung disease (HD) depends on the histopathological analysis of rectal biopsies. This review aims to define the best rectal biopsy technique. A systematic literature review and proportional meta-analysis of
article · May 2021
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Inpatient management of Hirschsprung’s associated enterocolitis treatment: the benefits of standardized care
Abstract Introduction Patients with Hirschsprung's disease (HSCR) remain at risk of developing Hirschsprung-associated enterocolitis (HAEC) after surgical intervention. As inpatient management remains variable, our institution implemented
article · Oct 2020
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The Extent of the Transition Zone in Hirschsprung's Disease
AbstractBackgroundRetained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.ObjectiveWe aimed to evaluate the extent of the histological transition zone in patients with Hirsc
article · May 2019
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Hirschsprung Disease Part II with Dr. Marc Levitt
The vast majority of Hirschsprung patients do extremely well post-pull-through with normal emptying and bowel control.
Marc Levitt3:29
Post-pull-through problem patients divide into two types: obstruction (failure to empty) and soiling.
Marc Levitt3:37
Enterocolitis after a well-done pull-through is common in babies because they have very tight sphincters and can keep them tight for many hours.
Marc Levitt4:25
After about age one, patients should learn to empty and relax their sphincters; enterocolitis after age one needs evaluation.
Marc Levitt4:59
Evaluation of recurrent enterocolitis involves a contrast study of the colon and an examination under anesthesia.
Marc Levitt5:20
Anatomic causes of post-pull-through obstruction include distal stricture, obstructing Soave cuff, atonic Duhamel pouch, twisted pull-through (up to 360°), and dilated distal segment.
Marc Levitt5:38
An obstructing Soave cuff is aganglionic outer rectal wall that can cause obstruction if not properly split or if it has fused or scarred.
Marc Levitt5:50
Pathologic cause of obstruction is pull-through to transition-zone bowel without healthy ganglion cells or with nerve roots larger than 40 microns.
Marc Levitt7:14
Acute enterocolitis treatment: IV hydration, IV metronidazole (most effective antibiotic), and aggressive rectal irrigations 2–3 times daily with 10–20 cc/kg saline via size 20–22 Foley catheter.
Marc Levitt8:20
If irrigations fail to relieve distention and improve the baby, the tube may not be reaching high enough or the patient may need an ileostomy.
Marc Levitt10:04
Metronidazole (Flagyl) has the same efficacy IV or PO because in both cases it is excreted in the bile.
Marc Levitt11:21
In a diverted colon with ileostomy, oral or IV Flagyl will not work for colitis because the drug exits via the ileostomy; vancomycin enemas are needed.
Marc Levitt11:32
Literature reports 15–20% of patients have an episode of enterocolitis within the first year after pull-through.
Marc Levitt12:13
On contrast study, look for cuff indentation in the presacral space; the pull-through should hug the sacrum, and anterior deviation suggests a space-occupying cuff.
Marc Levitt13:09
An obstructing Soave cuff can be felt on digital rectal exam under anesthesia as a rubbery thick rubber-band structure in the sacral hollow, outside the pull-through.
Marc Levitt16:34
Gastroenterologists used to intraluminal scope visualization may miss an obstructing cuff because it is outside the pull-through lumen.
Marc Levitt17:02
Full-thickness biopsy should be taken 1 cm above the dentate line and sent for permanent section to assess ganglion cell quality and nerve size; frozen section is not reliable for this critical redo diagnosis.
Marc Levitt17:30
Transition-zone bowel (ganglion cells present but hypertrophic nerves >40 microns) that is not functioning well requires redo pull-through.
Marc Levitt18:17
Redo pull-through for transition zone: transanal dissection preserving anal canal and dentate line, plus laparotomy or laparoscopy to mobilize to healthy ganglionated bowel, often requiring removal of retained sigmoid curve.
Marc Levitt18:27
For redo transanal dissection, hug the bowel to find the Swenson plane outside the original pull-through and outside the retained aganglionic cuff, which is an areolar space that has never been operated on.
Marc Levitt19:25
To remove an obstructing Soave cuff: dissect pull-through from cuff, then dissect cuff from Swenson plane, and excise the cuff posteriorly and laterally to break the ring—complete circumferential removal is unnecessary.
Marc Levitt20:31
Avoid aggressive anterior cuff dissection near the urethra and vagina; breaking the ring posterolaterally solves the obstruction.
Marc Levitt21:24
Myectomies that have been traditionally successful may have inadvertently cut the Soave cuff rather than the internal sphincter.
Expert opinionMarc Levitt21:51
Myectomy technique varies widely among surgeons; it is not standardized and can injure skeletal muscle, causing incontinence.
Expert opinionMarc Levitt22:09
Post-pull-through sphincter problems are relatively rare compared to anatomic causes like cuff, stricture, or transition-zone bowel.
Expert opinionMarc Levitt24:34
If all anatomic and pathologic causes are ruled out and the child still behaves obstructed, the sphincter must be the cause; this can be confirmed with anorectal manometry showing failure to relax.
Marc Levitt24:51
Sphincter achalasia (tight sphincter with failure to relax) is relatively rare after age one; most children learn sphincter coordination over time.
Marc Levitt25:19
Botox injection acts as a temporary myectomy and is preferred over permanent myectomy because it wears off as the child learns sphincter relaxation; a series of Botox injections is safer than permanent myectomy, which can cause incontinence.
Marc Levitt25:35
Coordinate Botox with laxatives: inject Botox, then at 4–8 weeks (as Botox wears off) start aggressive laxatives so the child learns appropriate bowel movement pattern with reduced sphincter tone.
Marc Levitt26:07
Botox may temporarily improve cuff obstruction if it migrates to the cuff level, but the patient will recur because the cuff must be surgically removed for long-term fix.
Marc Levitt26:45
Start the course
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