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Hirschsprung Disease: Diagnosis to Long-Term Management

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Children with persistent problems after Hirschsprung surgery should not be expected to improve over time; anatomic causes can almost always be identified and corrected. — Marc Levitt, Hirschsprung Disease – PediaCast 287 · 24:03

Hirschsprung
Welcome to an ePiPS special with Dr. Marc Levitt! I am here today at the 50th Anniversary of APSA in Boston discussing the diagnosis and management of Hirschspr
podcast22:45 · Jul 2026
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Hirschsprung Disease – PediaCast 287
Dr Marc Levitt joins Dr Mike in the PediaCast Studio for a comprehensive look at Hirschsprung Disease. We talk cause, symptoms, diagnosis, treatment, complicati
podcast38:55 · Jul 2026
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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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If a patient has recovered from enterocolitis and irrigations are going well with reliable family, it is reasonable to send them home for 1-2 months before definitive operation rather than operating at 2 weeks. — Jason Frischer, Colorectal Quiz: Episode 46 · 13:33

Classic Distal Disease3 items
The Colorectal Quiz Episode 4
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 Hi
podcast19:28 · Jul 2026
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Colorectal Quiz: Episode 46 - Hirschsprung's Disease
In this episode of the Colorectal Quiz, Drs. Marc Levitt, Jason Frischer, Kristy Rialon, and Lily Cheng explore the diagnostic and surgical challenges of Hirschsprung's disease, featuring a neonatal patient with Trisomy 21. Through a step-b
video29:52 · Jul 2026
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Colorectal Quiz: Episode 46
In this episode of the Colorectal Quiz, Drs. Marc Levitt, Jason Frischer, Kristy Rialon, and Lily Cheng explore the diagnostic and surgical challenges of Hirsch
podcast29:59 · Jul 2026
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Proximal & Extensive Disease2 items
The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique
video14:37 · Jul 2023Private
Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group
Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group - PubMed Clipboard, Search History, and several other advanc
article · Jul 2026
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Late Diagnosis & Special Situations2 items
Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease
Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A tr
article · Jul 2026
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Colorectal Quiz: Episode 44 - HD Frozen Section
In this episode of the Colorectal Quiz, Drs. Marc Levitt, Jason Frischer, and Martin Lacher dive into the intricacies of frozen sections and reoperative strategies in Hirschsprung disease. Using a fascinating case of a late-diagnosed Hirsch
video18:17 · Jul 2026
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Many pathologists are not measuring nerve caliber, and pediatric surgeons should demand that their pathologists do this to avoid doing a pull-through in the transition zone — Marc Levitt, Hirschsprung Disease Audience Q&A with Dr. Marc Levitt · 5:43

Sphincter Reconstruction2 items
Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes
Background: In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) h
article · Jan 2023
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A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease
Soiling and fecal incontinence are troubling complications which can occur after a pull-through for Hirschsprung disease. They can usually be improved with proper medical management, but in some cases are the result of an anatomic defect re
article · Jul 2026
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Long-Term Outcomes3 items
Hirschsprung Disease Part 2
Continuation of our previous podcast on Hirschsprung disease with Dr Marc Levitt that focuses on the evaluation and management of post-pull-through complication
podcast44:44 · Jul 2026
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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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Hirschsprung Disease Audience Q&A with Dr. Marc Levitt
Continuation of discussion on Hirschsprung disease with Dr. Marc Levitt with emphasis on the audience's questions .
podcast12:40 · Dec 2020
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The Duhamel is now really only appropriate for an ilio-Duhamel, although Levitt would still do an ilioanal. — Marc Levitt, The Colorectal Quiz Episode 21: The History of Hirschsprung Disease · 0:26

The Colorectal Quiz Episode 21: The History of Hirschsprung Disease
The treatment for Hirschsprung disease has undergone tremendous evolution over the past century. Here, Dr. Marc Levitt and Dr. Jason Frischer discuss the history of Hirschsprung disease and how the treatment has improved to what we know tod
video15:20 · Jul 2026
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The Colorectal Quiz Episode 21: The History of Hirschsprung Disease
The treatment for Hirschsprung disease has undergone tremendous evolution over the past century. Here, Dr. Marc Levitt and Dr. Jason Frischer discuss the histor
podcast15:56 · Jul 2026
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Contrast enema is accurate in identifying the transition zone about 90% of the time, so when doing surgery, you need to confirm under the microscope where the healthy bowel begins. — Marc Levitt, DrBeen Medical Lectures: Dr. Marc Levitt, MD Discusses Hirschsprung Disease · 22:09

DrBeen Medical Lectures: Dr. Marc Levitt, MD Discusses Hirschsprung Disease
Dr. Marc A. Levitt, MD Discusses Hirschsprung Disease on YouTube Live with Drbeen Medical Lectures. Link to DrBeen website: https://www.drbeen.com
video53:42 · Dec 2022
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Hirschsprung
Maternal magnesium sulfate or other tocolytic medications can cause significant ileus in the newborn that mimics Hirschsprung's disease.
Marc Levitt0:55
Hypothyroidism and opiate exposure (maternal addiction or overdose transmitted to baby) can present with abdominal distension and delayed meconium passage mimicking Hirschsprung's disease.
Marc Levitt0:55
Milk protein allergy in a fed baby can mimic Hirschsprung's disease on x-ray, and irrigations help even when it is not Hirschsprung's.
Marc Levitt0:55
Ten percent of patients with meconium plug have Hirschsprung's disease, so suction rectal biopsy should be performed even after successful plug passage.
Marc Levitt2:00
After meconium plug passage, the contrast study should be repeated because the initial study with plug present will show dilated rectosigmoid, but the repeat study will reveal the transition zone more clearly.
Marc Levitt3:00
In total colonic Hirschsprung's disease, there is often no obvious transition zone on contrast study.
Marc Levitt3:00
Suction rectal biopsy is the gold standard for diagnosing Hirschsprung's disease, and some centers proceed directly to biopsy without contrast study.
Marc Levitt4:00
Contrast study serves as a surgical GPS map of the colon anatomy and is preferred before operating, even though biopsy alone can rule out Hirschsprung's.
Expert opinionMarc Levitt4:00
The physiologic obstruction in Hirschsprung's disease leads to stasis, bacterial overgrowth, and bacterial translocation because mucosal integrity is abnormal.
Marc Levitt4:42
A constipated baby without Hirschsprung's disease will not develop enterocolitis because their mucosal integrity and IgA levels are normal.
Marc Levitt4:42
Down syndrome patients with Hirschsprung's disease have worse enterocolitis because they have a weaker immune barrier.
Marc Levitt4:42
Irrigation protocol: use a 20 French soft silicone Foley catheter with warm saline in 10-20cc aliquots, washing the inside of the colon by injecting, allowing drip-back, advancing the catheter a few centimeters, and repeating for up to 30 minutes.
Marc Levitt5:44
Irrigations should be performed two to three times per day and require a written protocol so nurses know exactly what is expected.
Marc Levitt5:44
If irrigations are performed correctly but do not reach the transition zone, the baby will remain distended and irrigations will not be effective.
Marc Levitt5:44
When irrigations fail despite correct technique, the surgeon must decide between a leveling colostomy (bringing dilated bowel to surface, ideally with frozen section confirmation) or an ileostomy with colonic biopsies.
Marc Levitt7:00
Ileostomy does not require intraoperative pathology at 3 AM, the baby will thrive, and full colonic pathology data can be obtained later, but it requires three operations instead of two.
Marc Levitt7:30
In regions where ileostomy management is difficult and babies can dehydrate quickly, ileostomy may not be appropriate.
Marc Levitt7:30
A leveling colostomy is usually accurate when placed in dilated bowel to the surgeon's eye, but the risk is opening a colostomy still in the transition zone.
Marc Levitt7:30
A loop ileostomy where both limbs are equal is inappropriate because stool will jump across and fill the aganglionic segment, causing continued distension and potential enterocolitis despite diversion.
Marc Levitt9:26
A Turnbull ileostomy (cutting the bowel completely on one side of the loop, intussuscepting it over a finger to create an end-stoma appearance with flat distal opening) or a divided ileostomy with proximal end brought up and distal limb tacked to it are both appropriate diversion techniques.
Marc Levitt9:26
Post-pull-through patients present with two distinct problems: obstruction (chronic distension, enterocolitis episodes, failure to thrive) or soiling (no distension, no enterocolitis, uncontrolled stooling).
Marc Levitt11:51
Anatomic causes of post-pull-through obstruction include inadequate Soave cuff (not cut enough, rolled up, or refused), twisted pull-through (180 or 360 degrees), Duhamel spur (two lumens not successfully joined), large Duhamel pouch reaching into pelvis, stricture, and transition zone pull-through.
Marc Levitt12:40
A Soave cuff feels like a rubbery circumferential ring outside the pull-through on digital exam.
Marc Levitt12:40
A twisted pull-through prevents the examining finger from entering the pelvis; on abdominal palpation you feel like you are hitting a wall.
Marc Levitt12:40
Contrast study may show a cuff by revealing extra presacral space on lateral view; the pull-through should hug the sacrum, and a cuff pushes it forward.
Marc Levitt12:40
In Duhamel patients, biopsy must be taken from the posterior wall because the anterior wall is the original aganglionic rectum.
Marc Levitt15:00
If anatomic causes and pathology are normal in an obstructed post-pull-through patient, the problem is non-relaxing sphincters, and empiric Botox is appropriate.
Marc Levitt15:00
Every child born with Hirschsprung's disease has 100% potential for bowel control because the continence mechanism is normal and the sphincters are strong, not weak.
Marc Levitt16:13
This is very different from anorectal malformation, where associated spinal problems, poor sacrum, or poor muscles can prevent continence despite a perfect operation.
Marc Levitt16:13
Overstretching of sphincters or starting transanal dissection too low (removing the dentate line) will cause iatrogenic fecal incontinence.
Marc Levitt17:10
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