# Hirschsprung Disease: Diagnosis to Long-Term Management — GCMD Library living collection

Updated: n/a · 18 episodes · 403 cited statements

## Episodes
### Diagnosis & Evaluation
- [Hirschsprung](https://qa.library.globalcastmd.com/watch/hirschsprung-13870) — podcast · 22:45 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-13870.md)
- [Hirschsprung Disease – PediaCast 287](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877) — podcast · 38:55 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877.md)
- [Hirschsprung Disease Part I with Marc Levitt](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311) — podcast · 59:20 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311.md)

### Surgical Techniques
- [The Colorectal Quiz Episode 4](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864) — podcast · 19:28 · [machine version](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864.md)
- [Colorectal Quiz: Episode 46 -  Hirschsprung's Disease](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821) — video · 29:52 · [machine version](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821.md)
- [Colorectal Quiz: Episode 46](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848) — podcast · 29:59 · [machine version](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848.md)
- [Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group](https://qa.library.globalcastmd.com/article/evaluation-and-management-of-total-colonic-hirschsprung-disease-a-comprehensive-13762) — article · [machine version](https://qa.library.globalcastmd.com/article/evaluation-and-management-of-total-colonic-hirschsprung-disease-a-comprehensive-13762.md)
- [Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease](https://qa.library.globalcastmd.com/article/transanal-only-swenson-like-pull-through-for-late-diagnosed-hirschsprung-disease-13806) — article · [machine version](https://qa.library.globalcastmd.com/article/transanal-only-swenson-like-pull-through-for-late-diagnosed-hirschsprung-disease-13806.md)
- [Colorectal Quiz: Episode 44 - HD Frozen Section](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823) — video · 18:17 · [machine version](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823.md)

### Postoperative Management & Complications
- [Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes](https://qa.library.globalcastmd.com/article/reconstructing-the-anal-sphincters-to-reverse-iatrogenic-overstretching-followin-6281) — article · [machine version](https://qa.library.globalcastmd.com/article/reconstructing-the-anal-sphincters-to-reverse-iatrogenic-overstretching-followin-6281.md)
- [A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease](https://qa.library.globalcastmd.com/article/a-technique-to-reconstruct-the-anal-sphincters-following-iatrogenic-stretching-r-13800) — article · [machine version](https://qa.library.globalcastmd.com/article/a-technique-to-reconstruct-the-anal-sphincters-following-iatrogenic-stretching-r-13800.md)
- [Hirschsprung Disease Part 2](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875) — podcast · 44:44 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875.md)
- [Hirschsprung Disease Part II with Dr. Marc Levitt](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310) — podcast · 44:38 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310.md)
- [Hirschsprung Disease Audience Q&A with Dr. Marc Levitt](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440) — podcast · 12:40 · [machine version](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440.md)

### History & Evolution
- [The Colorectal Quiz Episode 21: The History of Hirschsprung Disease](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818) — video · 15:20 · [machine version](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818.md)
- [The Colorectal Quiz Episode 21: The History of Hirschsprung Disease](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861) — podcast · 15:56 · [machine version](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861.md)

### Expert Discussions
- [DrBeen Medical Lectures: Dr. Marc Levitt, MD Discusses Hirschsprung Disease](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227) — video · 53:42 · [machine version](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227.md)

## Chapters
- [0:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=0) Introduction and recap of previous trauma episode (Ep 14)
- [2:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=140) Post-pull-through enterocolitis: presentation and pathophysiology (Ep 14)
- [5:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=320) Anatomic causes of post-pull-through obstruction (Ep 14)
- [8:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=500) Acute enterocolitis management protocol (Ep 14)
- [10:22](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=622) Evaluation of recurrent enterocolitis: contrast study and exam under anesthesia (Ep 14)
- [12:41](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=761) Examination under anesthesia technique and biopsy (Ep 14)
- [18:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1083) Surgical management of transition-zone pull-through (Ep 14)
- [20:13](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1213) Surgical management of obstructing Soave cuff (Ep 14)
- [23:43](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1423) Role of Botox in sphincter achalasia (Ep 14)
- [27:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1630) Management of twisted pull-through (Ep 14)
- [29:41](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1781) Redo of failed Duhamel pouch (Ep 14)
- [32:35](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1955) Evaluation of the soiling patient (Ep 14)
- [36:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2175) Four clinical scenarios in soiling patients (Ep 14)
- [42:39](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2559) Surgical options for incontinence and closing remarks (Ep 14)
- [0:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=0) Introduction and Appendicitis Review (Ep 3)
- [5:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=310) Initial Evaluation and Diagnosis of Hirschsprung Disease (Ep 3)
- [12:36](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=756) Pathologic Confirmation and Surgical Timing (Ep 3)
- [17:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1070) Historical Evolution of Surgical Techniques (Ep 3)
- [27:53](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1673) Current Surgical Approach: Transanal Swenson (Ep 3)
- [32:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1935) Technical Details: Exposure and Dissection Plane (Ep 3)
- [37:13](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2233) Laparoscopic Approach and Biopsy Technique (Ep 3)
- [47:56](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2876) Postoperative Management (Ep 3)
- [56:08](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3368) Special Circumstance: Hepatic Flexure Transition Zone (Ep 3)
- [0:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=0) Introduction and Question Setup (Ep 15)
- [1:34](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=94) Botox Mechanism and Primary Indications (Ep 15)
- [3:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=231) Borderline Age Group and Anatomic Workup (Ep 15)
- [7:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=470) Botox Technique Details and Volume Discussion (Ep 15)
- [9:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=549) Dilated Pull-Through Segments and Redo Surgery (Ep 15)
- [12:01](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=721) Closing (Ep 15)
- [0:01](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1) Introduction and Overview of Hirschsprung Disease (Ep 18)
- [4:12](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=252) Clinical Presentation and Differential Diagnosis (Ep 18)
- [9:55](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=595) Diagnostic Methods: Rectal Biopsy (Ep 18)
- [17:45](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1065) Pathology and Imaging Findings (Ep 18)
- [23:12](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1392) Surgical Treatment: Pull-Through Procedure (Ep 18)
- [28:15](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1695) Pre-operative Management and Enterocolitis (Ep 18)
- [40:34](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2434) Post-operative Complications and Botox Treatment (Ep 18)
- [44:15](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2655) Long-term Outcomes and Global Health Considerations (Ep 18)
- [0:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=0) Introduction and Overview of Historical Contributors (Ep 16)
- [1:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=60) Early Pioneers: Hirschsprung, Swenson, Yancey, and Suave (Ep 16)
- [4:06](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=246) Duhamel and Rabine Techniques (Ep 16)

## Statements
Every statement is attributed, typed, and timestamped; the link is the citation.
- Maternal magnesium sulfate or other tocolytic medications can cause significant ileus in the newborn that mimics Hirschsprung's disease. — Marc Levitt (clinical) [Ep 1 · 0:55](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=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 Levitt (clinical) [Ep 1 · 0:55](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=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 Levitt (clinical) [Ep 1 · 0:55](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=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 Levitt (epidemiological) [Ep 1 · 2:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=120)
- 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 Levitt (clinical) [Ep 1 · 3:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=180)
- In total colonic Hirschsprung's disease, there is often no obvious transition zone on contrast study. — Marc Levitt (clinical) [Ep 1 · 3:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=180)
- Suction rectal biopsy is the gold standard for diagnosing Hirschsprung's disease, and some centers proceed directly to biopsy without contrast study. — Marc Levitt (clinical) [Ep 1 · 4:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=240)
- 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. — Marc Levitt (opinion) [Ep 1 · 4:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=240)
- The physiologic obstruction in Hirschsprung's disease leads to stasis, bacterial overgrowth, and bacterial translocation because mucosal integrity is abnormal. — Marc Levitt (clinical) [Ep 1 · 4:42](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=282)
- A constipated baby without Hirschsprung's disease will not develop enterocolitis because their mucosal integrity and IgA levels are normal. — Marc Levitt (clinical) [Ep 1 · 4:42](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=282)
- Down syndrome patients with Hirschsprung's disease have worse enterocolitis because they have a weaker immune barrier. — Marc Levitt (clinical) [Ep 1 · 4:42](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=282)
- 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 Levitt (clinical) [Ep 1 · 5:44](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=344)
- Irrigations should be performed two to three times per day and require a written protocol so nurses know exactly what is expected. — Marc Levitt (clinical) [Ep 1 · 5:44](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=344)
- If irrigations are performed correctly but do not reach the transition zone, the baby will remain distended and irrigations will not be effective. — Marc Levitt (clinical) [Ep 1 · 5:44](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=344)
- 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 Levitt (clinical) [Ep 1 · 7:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=420)
- 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 Levitt (clinical) [Ep 1 · 7:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=450)
- In regions where ileostomy management is difficult and babies can dehydrate quickly, ileostomy may not be appropriate. — Marc Levitt (clinical) [Ep 1 · 7:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=450)
- 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 Levitt (clinical) [Ep 1 · 7:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=450)
- 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 Levitt (clinical) [Ep 1 · 9:26](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=566)
- 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 Levitt (clinical) [Ep 1 · 9:26](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=566)
- 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 Levitt (clinical) [Ep 1 · 11:51](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=711)
- 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 Levitt (clinical) [Ep 1 · 12:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=760)
- A Soave cuff feels like a rubbery circumferential ring outside the pull-through on digital exam. — Marc Levitt (clinical) [Ep 1 · 12:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=760)
- A twisted pull-through prevents the examining finger from entering the pelvis; on abdominal palpation you feel like you are hitting a wall. — Marc Levitt (clinical) [Ep 1 · 12:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=760)
- 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 Levitt (clinical) [Ep 1 · 12:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=760)
- In Duhamel patients, biopsy must be taken from the posterior wall because the anterior wall is the original aganglionic rectum. — Marc Levitt (clinical) [Ep 1 · 15:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=900)
- 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 Levitt (clinical) [Ep 1 · 15:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=900)
- 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 Levitt (clinical) [Ep 1 · 16:13](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=973)
- This is very different from anorectal malformation, where associated spinal problems, poor sacrum, or poor muscles can prevent continence despite a perfect operation. — Marc Levitt (clinical) [Ep 1 · 16:13](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=973)
- Overstretching of sphincters or starting transanal dissection too low (removing the dentate line) will cause iatrogenic fecal incontinence. — Marc Levitt (clinical) [Ep 1 · 17:10](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1030)
- Patients with damaged sphincters or removed dentate line need mechanical bowel management with enemas from below (Peristeen) or antegrade (Malone) because they lack continence potential. — Marc Levitt (clinical) [Ep 1 · 17:10](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1030)
- If the anus appears closed when the patient is awake, that usually indicates good sphincters. — Marc Levitt (clinical) [Ep 1 · 18:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1080)
- Anorectal manometry can objectively determine whether sphincters are intact. — Marc Levitt (clinical) [Ep 1 · 18:00](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1080)
- The vast majority of Hirschsprung's patients are constipated and need stimulant laxatives (senna or bisacodyl), not stool softeners. — Marc Levitt (clinical) [Ep 1 · 18:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1120)
- Adding water-soluble fiber to stimulant laxatives provides bulk to prevent watery stool, which is difficult to control, while maintaining propulsion effect. — Marc Levitt (clinical) [Ep 1 · 18:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1120)
- The goal of medical management is one to two well-formed stools per day, which usually translates into successful continence. — Marc Levitt (clinical) [Ep 1 · 18:40](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1120)
- Soiling patients who are withholding or have not learned to overcome their non-relaxing internal sphincter often need Botox to achieve successful potty training. — Marc Levitt (clinical) [Ep 1 · 19:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1170)
- Some Hirschsprung's patients have hypermotility and stool too frequently; stopping stimulant laxatives in these patients can achieve continence within two days. — Marc Levitt (clinical) [Ep 1 · 19:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1170)
- X-ray showing no stool accumulation in a child stooling five times per day indicates fast-moving colon (hypermotility). — Marc Levitt (clinical) [Ep 1 · 19:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1170)
- Contrast study distinguishes constipated (dilated colon) from hypermotile (non-dilated colon) patients. — Marc Levitt (clinical) [Ep 1 · 19:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1170)
- Hypermotile patients need constipating diet, water-soluble fiber, and occasionally loperamide to slow transit and achieve one to two formed stools per day. — Marc Levitt (clinical) [Ep 1 · 19:30](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1170)
- Nighttime soiling is typical because patients are totally dependent on voluntary external sphincter control, which they lose awareness of during sleep. — Marc Levitt (clinical) [Ep 1 · 20:48](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1248)
- Behavioral interventions for nighttime soiling include ensuring the child stools before bed and giving a small enema before bed to keep the rectum empty for eight hours. — Marc Levitt (clinical) [Ep 1 · 20:48](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1248)
- Malone appendicostomy is appropriate when rectal enemas are not tolerated, Peristeen is ineffective, or the family prefers not to use the rectal route. — Marc Levitt (clinical) [Ep 1 · 21:27](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1287)
- Malone is typically performed between ages 3.5 and 8-9 years, around the time of potty training when it becomes clear that antegrade flushes will be required. — Marc Levitt (clinical) [Ep 1 · 21:27](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1287)
- Most children should be in normal underwear (out of diapers) by age 3-4 years. — Marc Levitt (opinion) [Ep 1 · 21:27](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1287)
- The vast majority of Hirschsprung patients do extremely well post-pull-through with normal emptying and bowel control. — Marc Levitt (clinical) [Ep 14 · 3:29](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=209)
- Post-pull-through problem patients divide into two types: obstruction (failure to empty) and soiling. — Marc Levitt (clinical) [Ep 14 · 3:37](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=217)
- 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 Levitt (clinical) [Ep 14 · 4:25](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=265)
- After about age one, patients should learn to empty and relax their sphincters; enterocolitis after age one needs evaluation. — Marc Levitt (clinical) [Ep 14 · 4:59](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=299)
- Evaluation of recurrent enterocolitis involves a contrast study of the colon and an examination under anesthesia. — Marc Levitt (clinical) [Ep 14 · 5:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=320)
- 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 Levitt (clinical) [Ep 14 · 5:38](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=338)
- 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 Levitt (clinical) [Ep 14 · 5:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=350)
- Pathologic cause of obstruction is pull-through to transition-zone bowel without healthy ganglion cells or with nerve roots larger than 40 microns. — Marc Levitt (clinical) [Ep 14 · 7:14](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=434)
- 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 Levitt (clinical) [Ep 14 · 8:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=500)
- 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 Levitt (clinical) [Ep 14 · 10:04](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=604)
- Metronidazole (Flagyl) has the same efficacy IV or PO because in both cases it is excreted in the bile. — Marc Levitt (clinical) [Ep 14 · 11:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=681)
- 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 Levitt (clinical) [Ep 14 · 11:32](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=692)
- Literature reports 15–20% of patients have an episode of enterocolitis within the first year after pull-through. — Marc Levitt (epidemiological) [Ep 14 · 12:13](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=733)
- 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 Levitt (clinical) [Ep 14 · 13:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=789)
- 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 Levitt (clinical) [Ep 14 · 16:34](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=994)
- Gastroenterologists used to intraluminal scope visualization may miss an obstructing cuff because it is outside the pull-through lumen. — Marc Levitt (clinical) [Ep 14 · 17:02](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1022)
- 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 Levitt (clinical) [Ep 14 · 17:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1050)
- Transition-zone bowel (ganglion cells present but hypertrophic nerves >40 microns) that is not functioning well requires redo pull-through. — Marc Levitt (clinical) [Ep 14 · 18:17](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1097)
- 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 Levitt (clinical) [Ep 14 · 18:27](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1107)
- 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 Levitt (clinical) [Ep 14 · 19:25](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1165)
- 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 Levitt (clinical) [Ep 14 · 20:31](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1231)
- Avoid aggressive anterior cuff dissection near the urethra and vagina; breaking the ring posterolaterally solves the obstruction. — Marc Levitt (clinical) [Ep 14 · 21:24](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1284)
- Myectomies that have been traditionally successful may have inadvertently cut the Soave cuff rather than the internal sphincter. — Marc Levitt (opinion) [Ep 14 · 21:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1311)
- Myectomy technique varies widely among surgeons; it is not standardized and can injure skeletal muscle, causing incontinence. — Marc Levitt (opinion) [Ep 14 · 22:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1329)
- Post-pull-through sphincter problems are relatively rare compared to anatomic causes like cuff, stricture, or transition-zone bowel. — Marc Levitt (opinion) [Ep 14 · 24:34](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1474)
- 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 Levitt (clinical) [Ep 14 · 24:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1491)
- Sphincter achalasia (tight sphincter with failure to relax) is relatively rare after age one; most children learn sphincter coordination over time. — Marc Levitt (clinical) [Ep 14 · 25:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1519)
- 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 Levitt (clinical) [Ep 14 · 25:35](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1535)
- 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 Levitt (clinical) [Ep 14 · 26:07](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1567)
- 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 Levitt (clinical) [Ep 14 · 26:45](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1605)
- Anorectal manometry can measure the length of high-tone zone: 1 cm of high tone is sphincter achalasia; 3–4 cm is sphincter plus obstructing cuff. — Marc Levitt (clinical) [Ep 14 · 27:17](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1637)
- Twisted pull-through: dissect in Swenson plane around pull-through to peritoneal reflection, then laparotomy (preferred over laparoscopy due to adhesions) to mobilize left colon, preserve sigmoid arcade, deliver bowel into abdomen, untwist, and re-pull-through. — Marc Levitt (clinical) [Ep 14 · 28:01](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1681)
- Redo of failed Duhamel pouch is the hardest Hirschsprung redo due to pelvic fibrosis from the stapled anastomosis. — Marc Levitt (opinion) [Ep 14 · 30:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1818)
- Duhamel redo technique: transanal dissection to separate pull-through from original rectum, then open abdominal approach with St. Mark's retractor for deep pelvic dissection, remove entire pouch, and convert to Swenson pull-through. — Marc Levitt (clinical) [Ep 14 · 31:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1860)
- All Hirschsprung patients should be able to empty spontaneously and be clean; the concept that they will eventually get better as teenagers is wrong. — Marc Levitt (opinion) [Ep 14 · 33:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1995)
- Hirschsprung patients are born with a normal anal canal and normal sphincters; if anything, their sphincters are too good (too tight). — Marc Levitt (clinical) [Ep 14 · 33:48](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2028)
- No Hirschsprung patient is born with a missing anal canal or weak sphincter; soiling due to these defects is iatrogenic from surgery. — Marc Levitt (clinical) [Ep 14 · 34:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2043)
- Iatrogenic incontinence occurs when the surgeon starts transanal dissection too low and invades the dentate line, or overstretches the sphincter during dissection. — Marc Levitt (clinical) [Ep 14 · 34:28](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2068)
- Laparoscopy-assisted pull-through avoids deep transanal work and reduces risk of sphincter overstretching compared to aggressive transanal dissection. — Marc Levitt (opinion) [Ep 14 · 35:04](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2104)
- Soiling patient evaluation: determine original operation type (Soave, Duhamel, Swenson), assess sphincter patency (visual, digital, manometry), and assess dentate-line integrity during EUA. — Marc Levitt (clinical) [Ep 14 · 35:36](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2136)
- Perform digital rectal exam as the patient goes to sleep (before full muscle relaxation) to assess sphincter squeeze; many soiling patients cannot squeeze. — Marc Levitt (clinical) [Ep 14 · 36:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2179)
- Contrast study in soiling patients: narrow non-dilated pull-through suggests hypermotility; dilated pull-through suggests hypomotility. — Marc Levitt (clinical) [Ep 14 · 37:06](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2226)
- Four soiling scenarios: (1) Good sphincter/anal canal + narrow colon = hypermotile, treat with loperamide, constipating diet, fiber; (2) Good sphincter/anal canal + dilated colon = hypomotile, treat with laxatives (bridge enemas until medical regimen works); (3) Lost sphincter/anal canal + narrow colon = hypermotile without continence capacity, treat with small-volume enema plus hypermotility agents; (4) Lost sphincter/anal canal + dilated colon = no continence capacity, treat with larger-volume enema. — Marc Levitt (clinical) [Ep 14 · 37:43](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2263)
- Hirschsprung soiling differs from anorectal malformation incontinence: Hirschsprung patients were born with good anatomy, so incontinence must be iatrogenic from surgery. — Marc Levitt (clinical) [Ep 14 · 39:12](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2352)
- Patients with intact sphincter and anal canal have capacity for bowel control and should achieve normal continence with appropriate medical management (laxatives or constipating agents). — Marc Levitt (clinical) [Ep 14 · 40:35](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2435)
- Capacity for bowel control is determined by intact sphincters and anal canal; if these are destroyed, the patient may not have the ability for voluntary bowel control. — Marc Levitt (clinical) [Ep 14 · 41:13](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2473)
- Patients without capacity for bowel control (destroyed sphincter/anal canal) may need Malone appendicostomy or cecostomy for antegrade enema administration. — Marc Levitt (clinical) [Ep 14 · 42:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2550)
- With methodical evaluation (contrast enema and examination under anesthesia), there is no problematic Hirschsprung patient that cannot be figured out and improved. — Marc Levitt (opinion) [Ep 14 · 43:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2580)
- The best treatment for Hirschsprung disease is irrigations using a large bore tube (20 French Foley) with warm saline, instilling 10-20 cc aliquots at a time and allowing fluid mixed with stool to drain back. — Marc Levitt (clinical) [Ep 3 · 8:43](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=523)
- It is very rare that Hirschsprung disease is a surgical emergency, but if you don't irrigate and overcome the distal obstruction, it will become an emergency. — Marc Levitt (clinical) [Ep 3 · 8:55](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=535)
- For rectal biopsy in suspected Hirschsprung disease, you must be at least 1 centimeter in from the dentate line. If you biopsy too close to the dentate line, everyone has an aganglionic segment there and you could get the wrong answer. — Marc Levitt (clinical) [Ep 3 · 12:38](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=758)
- The pathologist must report both the absence of ganglion cells AND the presence of hypertrophic nerves. The absence of ganglion cells alone is not Hirschsprung disease - that could be a biopsy taken too low. — Marc Levitt (clinical) [Ep 3 · 13:23](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=803)
- Hirschsprung disease has an immune component and the lining of the bowel (mucosa) is much more susceptible to bacterial translocation. Stasis in a Hirschsprung patient leads to bacterial translocation and a very sick baby from bacteremia. — Marc Levitt (clinical) [Ep 3 · 14:59](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=899)
- If irrigations are not working and the baby is ill, you need to divert. The recommendation is to divert in the ileum rather than doing a leveling colostomy, because frozen section can be inaccurate, particularly as you move higher in the colon. — Marc Levitt (clinical) [Ep 3 · 15:42](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=942)
- The Swenson operation involved full-thickness transabdominal dissection down below the peritoneal reflection. The operation was often done incorrectly with perirectal dissection done too wide, leading to fecal incontinence, urinary incontinence, sexual problems, and impotence. — Marc Levitt (clinical) [Ep 3 · 18:14](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1094)
- The Suave operation used a mucosal dissection to keep the outer rectal wall intact and avoid injury. The original Suave would leave the colon coming through the anus for a week before doing the coloanal anastomosis; Dr. Scott Boley modified it to do everything in one stage. — Marc Levitt (clinical) [Ep 3 · 19:28](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1168)
- The Duhamel operation leaves the original rectum in place, removes the aganglionic portion from the peritoneal reflection, and pulls ganglionic bowel in a retrorectal position, connecting the two lumens with a stapler. — Marc Levitt (clinical) [Ep 3 · 20:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1218)
- Of the four classic procedures (Swenson, Suave, Duhamel, Rabine), only the Swenson actually leaves behind virtually no Hirschsprung tissue. The others leave behind outer rectal wall or original rectum. Many patients did well despite this because ganglionic bowel, if it's good, can overcome a lot. — Marc Levitt (clinical) [Ep 3 · 21:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1278)
- Dr. Henrys in the Philippines pioneered primary pull-through operations (without initial colostomy) out of necessity because patients would never return to clinic due to social stigma against colostomies. — Marc Levitt (clinical) [Ep 3 · 22:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1339)
- Keith Jorgeson applied laparoscopic skills to Hirschsprung disease, doing the colonic work laparoscopically with a Suave dissection from above. Luis de la Torre and Jack Langer developed the transanal approach, starting the dissection from below. — Marc Levitt (clinical) [Ep 3 · 23:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1401)
- The current preferred approach is a transanal Swenson (full-thickness dissection). It is the purest operation, leaving behind no Hirschsprung except the very bottom just above the dentate line. If you find the right plane, it's elegant and bloodless. — Marc Levitt (opinion) [Ep 3 · 25:34](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1534)
- Dr. Orvar Swenson, who recently died at age 105, maintained that the Swenson operation was good all along and people just weren't doing it right, which gave it a bad reputation. — Marc Levitt (clinical) [Ep 3 · 26:23](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1583)
- Even Suave enthusiasts are making shorter and shorter cuffs over time. Jorgeson's original laparoscopic Suave recommended a 5 cm cuff; nowadays Langer and de la Torre do 1-1.5 cm cuffs, essentially approaching a Swenson. — Marc Levitt (clinical) [Ep 3 · 27:06](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1626)
- Laparoscopy is appropriate for all cases to find the transition zone level. A transanal-only approach is only appropriate when you have a very reachable, obvious transition zone at mid-sigmoid that you can comfortably reach transanally. — Marc Levitt (opinion) [Ep 3 · 28:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1730)
- There is significant morbidity from an overly aggressive transanal-only approach trying to reach the transition zone without going into the abdomen. If you're too aggressive transanally trying to visualize the dissection, you would have been better off with laparoscopic dissection. — Marc Levitt (clinical) [Ep 3 · 29:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1758)
- Total colonic Hirschsprung patients present differently: the diagnosis isn't made right away, the contrast study is not typical, and irrigations don't go well. These patients need laparoscopy first to find the biopsy location. — Marc Levitt (clinical) [Ep 3 · 31:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1878)
- The biggest technical problems are: (1) surgeons don't give themselves good exposure and start dissection too low, injuring or resecting the dentate line, or (2) they give very aggressive exposure and overstretch the sphincters. — Marc Levitt (clinical) [Ep 3 · 34:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2049)
- Proper technique requires marking 1 cm proximal to the dentate line with a purple mark and placing 5-0 silk stitches circumferentially at that level. This preserves the anal canal, dentate line, plus an additional 1 cm of columnar epithelium before starting dissection. — Marc Levitt (clinical) [Ep 3 · 35:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2130)
- By definition, you're leaving behind 1 cm of columnar epithelium that is Hirschsprung disease, plus the internal sphincter which has a problem with relaxation. However, good ganglionic bowel is able to overcome that, and the baby eventually learns to relax the sphincter. — Marc Levitt (clinical) [Ep 3 · 36:39](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2199)
- The rectum doesn't really have a mesentery; the rectum's blood supply is intramural. As you get higher and reach the peritoneal reflection, that's when you start to see sigmoidal vessels where rectum transitions to sigmoid. — Marc Levitt (clinical) [Ep 3 · 38:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2299)
- The anterior rectum frees up much quicker than the posterior rectum. Break into the peritoneal reflection anteriorly first. In transanal-only cases, you can pull the sigmoid out anteriorly, do a full-thickness biopsy, and send it while continuing the posterior dissection. — Marc Levitt (clinical) [Ep 3 · 38:45](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2325)
- For laparoscopic biopsies through the umbilicus, a seromuscular biopsy (without violating mucosa) may show ganglion cells in the seromuscular layer but miss hypertrophic nerves in the submucosa. If using this technique, send a full-thickness biopsy later to confirm the level is appropriate. — Marc Levitt (clinical) [Ep 3 · 40:56](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2456)
- Pathologists must report not only ganglion cells but also the quality of nerves with actual micron measurements. Nerves should be 40 microns or less. Anything bigger than 40 microns is transition zone bowel. — Marc Levitt (clinical) [Ep 3 · 44:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2643)
- The concept of 'go 5 cm above the transition zone' is inaccurate. Transition zone is a spectrum - some are 10 cm, some are 3 cm. You need confirmatory biopsy with ganglion cell information and nerve quality measured in microns. — Marc Levitt (clinical) [Ep 3 · 44:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2660)
- It's preferable to take the IMA, preserve the arcade, and have the left colon and sigmoid nice and straight down into the perineum. This makes for an easy-to-irrigate baby. Many patients have not had enough of a pull-through with the entire sigmoid loop still there, requiring redo to remove more. — Marc Levitt (clinical) [Ep 3 · 45:57](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2757)
- For anything proximal to the splenic flexure, do colonic biopsies and an ileostomy and wait, because frozen section has been notoriously fraught with errors in those cases. There is no urgency. Another option is to take biopsies, quit without diverting, and return 3-4 days later for pull-through with permanent section results. — Marc Levitt (clinical) [Ep 3 · 47:02](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2822)
- Postoperatively, wait until the belly is absolutely soft and flat with bowel function before feeding. Get an X-ray because abdominal distention is sometimes subclinical. This usually takes 3-4 days. Feeding too early risks the baby going home distended and returning with enterocolitis. — Marc Levitt (clinical) [Ep 3 · 53:23](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3203)
- Routine postoperative management: at one month, check the anus with Hagar dilators (not finger). Most babies need calibration rather than true dilation. The stimulation of passing the Hagar has value to help the baby more successfully empty. — Marc Levitt (clinical) [Ep 3 · 54:39](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3279)
- Flagyl is only used to treat enterocolitis, not routinely postoperatively. Give a pre-op dose of second-generation cephalosporin and maybe 2 post-op doses. — Marc Levitt (clinical) [Ep 3 · 55:52](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3352)
- Routine irrigations are only done postoperatively if the baby develops significant distention, which is rare. However, for total colonic patients after ileoanal pull-through, send all patients home on irrigations for 3 months. — Marc Levitt (clinical) [Ep 3 · 56:31](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3391)
- For hepatic flexure transition zone (rare), open the patient rather than laparoscopy. Take down the entire right colon, recognize the ileocolic vessel and how it feeds the vessel paralleling the right colon. Often need to take the right colic artery. De-rotate the colon so cecum is at the hepatic liver bed, then do pull-through down the right side of abdomen. — Marc Levitt (clinical) [Ep 3 · 57:05](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3425)
- If bringing de-rotated colon down the left side of abdomen, must mobilize the ligament of Treitz so the mesenteric vessel is not draped across the third portion of duodenum, which can cause duodenal obstruction. — Marc Levitt (clinical) [Ep 3 · 58:02](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3482)
- The vast majority of Hirschsprung patients do extremely well after pull-through with normal emptying and bowel control. — Marc Levitt (clinical) [Ep 13 · 3:28](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=208)
- Problem patients divide into two types: obstruction patients (cannot empty) and soiling patients (lack control). — Marc Levitt (clinical) [Ep 13 · 3:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=230)
- Enterocolitis after well-done pull-through is common in babies because they have very tight sphincters capable of staying tight for many hours. — Marc Levitt (clinical) [Ep 13 · 4:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=260)
- After about age one, patients should learn to empty and relax sphincters with normal bowel movement pattern; persistent enterocolitis after age one requires evaluation. — Marc Levitt (clinical) [Ep 13 · 4:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=290)
- Evaluation of post-pull-through obstruction involves contrast study of colon and examination under anesthesia. — Marc Levitt (clinical) [Ep 13 · 5:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=315)
- Anatomic causes of post-pull-through obstruction include: distal stricture, obstructing cuff, Duhamel pouch dysfunction, twisted pull-through (up to 360 degrees), and dilated distal segment. — Marc Levitt (clinical) [Ep 13 · 5:38](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=338)
- Pathologic cause of obstruction: pull-through not done to ganglionated bowel with normal-sized nerves (transition zone bowel with nerves >40 microns will not function). — Marc Levitt (clinical) [Ep 13 · 7:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=420)
- Treatment for enterocolitis: rule out stricture with Hagar dilators (not finger in babies), provide IV hydration and IV metronidazole, perform rectal irrigations 2-3 times daily. — Marc Levitt (clinical) [Ep 13 · 8:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=499)
- Irrigation technique: 10-20 cc/kg saline through size 20-22 Foley catheter, instill and let drip out, move tube to wash colon interior. — Marc Levitt (clinical) [Ep 13 · 9:37](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=577)
- Metronidazole has same efficacy IV or PO because both routes depend on biliary excretion into colon. — Marc Levitt (clinical) [Ep 13 · 11:07](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=667)
- In diverted colon (with ileostomy), flagyl will not work for colitis because drug exits via ileostomy; vancomycin enemas are needed. — Marc Levitt (clinical) [Ep 13 · 11:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=690)
- Literature reports 15-20% of patients have enterocolitis episode within first year after pull-through; after one year, persistent enterocolitis warrants anatomic or pathologic investigation. — Marc Levitt (epidemiological) [Ep 13 · 12:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=741)
- On contrast study, look for cuff as indentation in presacral space (pull-through should hug sacrum); cuff can divert pull-through forward. — Marc Levitt (clinical) [Ep 13 · 12:58](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=778)
- Soave cuff is outside the pull-through lumen and cannot be detected intraluminally with endoscopy; must be felt on digital exam or seen on lateral contrast view. — Marc Levitt (clinical) [Ep 13 · 15:18](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=918)
- On EUA, use fifth digit to feel along sacral hollow for rubbery thick rubber-band structure (cuff) outside the pull-through. — Marc Levitt (clinical) [Ep 13 · 16:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1000)
- Biopsy should be taken above dentate line by about 1 cm and sent for permanent section (not frozen) to assess ganglion cells and nerve size in redo situations. — Marc Levitt (clinical) [Ep 13 · 17:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1040)
- Transition zone pull-through (ganglion cells present but hypertrophic nerves) in symptomatic patient requires redo pull-through to healthy ganglionated bowel. — Marc Levitt (clinical) [Ep 13 · 18:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1101)
- Redo pull-through approach: prone and supine, transanal dissection preserving anal canal and dentate line, full-thickness dissection of pull-through, removal of cuff, may require laparotomy/laparoscopy for mobilization to healthy bowel. — Marc Levitt (clinical) [Ep 13 · 19:19](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1159)
- For persistent cuff: dissect between bowel and cuff, then dissect cuff in Swenson plane; remove posterior-lateral ring (not entire circumference) to break obstruction while avoiding anterior injury to urethra/vagina. — Marc Levitt (clinical) [Ep 13 · 20:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1230)
- Successful myectomies likely work by cutting the cuff rather than internal sphincter; myectomy technique varies widely between surgeons. — Marc Levitt (opinion) [Ep 13 · 21:49](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1309)
- Myectomy can cause permanent incontinence by damaging skeletal muscle; Dr. Levitt strongly argues against myectomy in favor of targeted cuff removal or Botox. — Marc Levitt (opinion) [Ep 13 · 22:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1360)
- Post-pull-through sphincter problems are relatively rare compared to anatomic causes; most obstruction is not sphincter-related. — Marc Levitt (clinical) [Ep 13 · 23:58](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1438)
- Anorectal manometry can confirm sphincter dysfunction (failure to relax) and distinguish sphincter-only high tone (1 cm) from sphincter-plus-cuff (3-4 cm of high tone). — Marc Levitt (clinical) [Ep 13 · 26:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1600)
- Botox acts as temporary myectomy; preferred over permanent myectomy because it wears off as children learn sphincter coordination (typically by 4-8 weeks with laxative support). — Marc Levitt (clinical) [Ep 13 · 24:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1480)
- Botox can temporarily improve cuff obstruction if injection migrates to cuff level, but patient will not achieve long-term improvement without cuff removal. — Marc Levitt (clinical) [Ep 13 · 26:45](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1605)
- For twisted pull-through: dissect in Swenson plane to peritoneal reflection, usually via open laparotomy (not laparoscopy) due to adhesions, preserve sigmoid arcade, deliver bowel into abdomen and re-pull through untwisted. — Marc Levitt (clinical) [Ep 13 · 27:55](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1675)
- Redo Duhamel is the hardest Hirschsprung reoperation due to pelvic fibrosis from stapled connection of two lumens. — Marc Levitt (opinion) [Ep 13 · 29:49](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1789)
- Redo Duhamel technique: start transanal, dissect around both pull-through (posterior) and original rectum (anterior), go to abdomen for deep pelvic dissection with St. Mark's retractor, connect planes, remove pouch and mated segment, redo as Swenson. — Marc Levitt (clinical) [Ep 13 · 31:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1860)
- All Hirschsprung patients should be able to empty spontaneously and be clean; concept that patients improve over many years into teenage years is wrong. — Marc Levitt (opinion) [Ep 13 · 32:58](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=1978)
- By definition, Hirschsprung patients are born with normal anal canal and normal (if anything, too strong) sphincters; no Hirschsprung patient is born with missing anal canal or weak sphincter. — Marc Levitt (clinical) [Ep 13 · 33:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2020)
- Soiling Hirschsprung patients with lost anal canal or weak sphincters have iatrogenic injury; causes are starting transanal dissection too low (invading dentate line) or overstretching during dissection. — Marc Levitt (clinical) [Ep 13 · 34:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2070)
- Overstretching was common with aggressive transabdominal deep pelvic dissection; now recurring with too-aggressive transanal dissection; laparoscopy avoids deep transanal work. — Marc Levitt (clinical) [Ep 13 · 35:20](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2120)
- Soiling evaluation: determine original surgery type, assess sphincter patency (visual, digital, manometry), assess dentate line integrity; perform digital exam as patient goes to sleep to test squeeze. — Marc Levitt (clinical) [Ep 13 · 36:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2160)
- Contrast study in soiling patient serves as 'poor man's colonic manometry': narrow non-dilated pull-through suggests hypermotility; dilated pull-through suggests hypomotility. — Marc Levitt (clinical) [Ep 13 · 37:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2220)
- Four soiling scenarios: (1) intact anatomy + non-dilated colon (hypermotile) → slow with loperamide, constipating diet, water-soluble fiber; (2) intact anatomy + dilated colon (hypomotile) → laxatives, possibly bridge enemas; (3) destroyed anatomy + non-dilated colon → small enemas + anti-motility; (4) destroyed anatomy + dilated colon → large enemas. — Marc Levitt (clinical) [Ep 13 · 37:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2270)
- Capacity for bowel control is determined by intact sphincters and anal canal; patients with capacity can usually be managed medically; those without capacity need enema programs. — Marc Levitt (clinical) [Ep 13 · 39:47](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2387)
- Surgical management (Malone appendicostomy or cecostomy) is only for patients without capacity for bowel control. — Marc Levitt (clinical) [Ep 13 · 42:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2550)
- Methodical evaluation with contrast study and examination under anesthesia reveals solvable pathology in most problematic pull-through patients. — Marc Levitt (opinion) [Ep 13 · 43:45](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-2-13875?t=2625)
- Hirschsprung disease affects approximately 1 in 5,000 children. — Marc Levitt (epidemiological) [Ep 2 · 2:31](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=151)
- In Hirschsprung disease, nerve ganglia are absent in the distal colon, causing the affected segment to remain contracted and unable to relax. — Marc Levitt (clinical) [Ep 2 · 5:58](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=358)
- The proximal colon dilates as it attempts to push stool through the contracted distal segment. — Marc Levitt (clinical) [Ep 2 · 6:48](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=408)
- Stasis of stool in the dilated proximal colon allows bacterial overgrowth, creating a 'swamp' that can lead to enterocolitis. — Marc Levitt (clinical) [Ep 2 · 6:48](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=408)
- Girls are slightly more commonly affected by Hirschsprung disease, but the difference is not dramatic; approximately half of patients are boys and half are girls. — Marc Levitt (epidemiological) [Ep 2 · 7:40](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=460)
- There are no racial or ethnic differences in Hirschsprung disease incidence. — Marc Levitt (epidemiological) [Ep 2 · 8:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=489)
- Down syndrome is associated with a higher incidence of Hirschsprung disease than the general population. — Marc Levitt (epidemiological) [Ep 2 · 8:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=489)
- Hirschsprung disease shows familial clustering, with documented cases spanning three generations (grandparent, parent, child). — Marc Levitt (epidemiological) [Ep 2 · 8:45](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=525)
- Nerve ganglia normally migrate from proximal to distal during bowel development; in Hirschsprung disease, this migration is incomplete, always affecting the distal bowel with no skip areas. — Marc Levitt (clinical) [Ep 2 · 10:12](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=612)
- Most infants with Hirschsprung disease fail to pass meconium in the first 24 hours of life; suspicion increases if no stool has passed by 48 hours. — Marc Levitt (clinical) [Ep 2 · 11:05](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=665)
- Affected infants develop abdominal distention, irritability, and vomiting. — Marc Levitt (clinical) [Ep 2 · 11:05](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=665)
- Infants with very short-segment Hirschsprung disease may not present in the newborn period and can eat and stool, though never well, remaining chronically distended. — Marc Levitt (clinical) [Ep 2 · 12:12](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=732)
- Older children with undiagnosed Hirschsprung disease typically show failure to thrive, chronic abdominal distention, and episodes of diarrhea (which are actually enterocolitis). — Marc Levitt (clinical) [Ep 2 · 13:01](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=781)
- Anorectal malformation (imperforate anus) is a key differential diagnosis; careful inspection of the perineum is essential to confirm an anal opening exists. — Marc Levitt (clinical) [Ep 2 · 14:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=861)
- Maternal magnesium sulfate (used to slow contractions during difficult delivery) can cause transient bowel dysmotility in newborns lasting several days. — Marc Levitt (clinical) [Ep 2 · 14:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=861)
- Hypothyroidism and maternal narcotic exposure can both cause severe neonatal bowel dysmotility. — Marc Levitt (clinical) [Ep 2 · 14:21](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=861)
- Cystic fibrosis can present with neonatal bowel obstruction and typically has a characteristic X-ray appearance. — Marc Levitt (clinical) [Ep 2 · 15:33](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=933)
- Diagnosis begins with plain abdominal X-ray showing colonic distention, followed by contrast enema demonstrating a narrow distal segment with proximal dilation. — Marc Levitt (clinical) [Ep 2 · 16:11](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=971)
- Definitive diagnosis requires rectal biopsy showing both absence of ganglion cells AND hypertrophied nerve trunks; both criteria are necessary. — Marc Levitt (clinical) [Ep 2 · 16:11](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=971)
- Water-soluble contrast is preferred over barium for contrast enemas in suspected Hirschsprung disease. — Marc Levitt (clinical) [Ep 2 · 16:11](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=971)
- Initial management requires rectal catheter insertion to decompress the colon, allowing gas and liquid stool to rush out. — Marc Levitt (clinical) [Ep 2 · 18:14](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1094)
- Rectal irrigations (not enemas) must be performed repeatedly to wash the colonic lining and prevent bacterial overgrowth; irrigations involve continuous fluid exchange, not simple instillation. — Marc Levitt (clinical) [Ep 2 · 18:14](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1094)
- Historically, Hirschsprung disease required three operations over six months: colostomy creation, pull-through procedure, and colostomy closure. — Marc Levitt (clinical) [Ep 2 · 20:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1215)
- Modern surgical technique allows the entire operation to be performed transanally with no abdominal incisions, sometimes with laparoscopic assistance. — Marc Levitt (clinical) [Ep 2 · 20:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1215)
- Modern single-stage repair can be performed in the first week of life, with hospital discharge several days later. — Marc Levitt (clinical) [Ep 2 · 21:08](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1268)
- Enterocolitis in Hirschsprung disease is diarrhea occurring proximal to the obstruction, invisible to clinicians because stool cannot exit the anus. — Marc Levitt (clinical) [Ep 2 · 21:57](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1317)
- Fluid shifts from the infant's body into the obstructed colon cause severe dehydration without visible diarrhea. — Marc Levitt (clinical) [Ep 2 · 21:57](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1317)
- Several infants die annually in the United States from Hirschsprung enterocolitis; mortality is higher in developing countries. — Marc Levitt (epidemiological) [Ep 2 · 21:57](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1317)
- Inflamed colonic mucosa allows bacterial translocation into the bloodstream, causing sepsis. — Marc Levitt (clinical) [Ep 2 · 23:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1390)
- Bowel perforation can occur in the most distended area if enterocolitis progresses, though this is extremely rare. — Marc Levitt (clinical) [Ep 2 · 23:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1390)
- Rectal irrigations performed by skilled neonatal nurses break the enterocolitis cycle and prevent progression to perforation and sepsis. — Marc Levitt (clinical) [Ep 2 · 23:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1390)
- An elegantly performed operation should result in normal bowel emptying and 100% normal bowel function. — Marc Levitt (opinion) [Ep 2 · 24:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1443)
- Imperfect initial surgery can leave anatomic problems causing either obstructive symptoms or fecal incontinence. — Marc Levitt (clinical) [Ep 2 · 24:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1443)
- 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 (opinion) [Ep 2 · 24:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1443)
- Re-evaluation of children with persistent problems should include contrast study, rectal biopsy to confirm adequate bowel was used, and surgical examination of the pull-through segment. — Marc Levitt (clinical) [Ep 2 · 24:03](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1443)
- Even after anatomically perfect operations, some children have mild constipation requiring laxatives and dietary modifications, similar to functional constipation management. — Marc Levitt (clinical) [Ep 2 · 27:05](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1625)
- Current research priorities include identifying the specific genetic mutations causing Hirschsprung disease. — Marc Levitt (clinical) [Ep 2 · 27:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1671)
- A key research question is why the colonic mucosa in Hirschsprung patients is uniquely susceptible to bacterial translocation and enterocolitis, unlike normal children with constipation. — Marc Levitt (clinical) [Ep 2 · 27:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1671)
- Research is ongoing to understand why anatomically normal colon after successful repair sometimes still has slow motility. — Marc Levitt (clinical) [Ep 2 · 27:51](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1671)
- The Center for Colorectal and Pelvic Reconstruction unifies four traditionally independent teams: colorectal surgery, urology, gynecology, and gastroenterology/motility. — Marc Levitt (clinical) [Ep 2 · 29:11](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1751)
- The center has treated patients from all 50 U.S. states and 88 countries. — Marc Levitt (clinical) [Ep 2 · 32:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1970)
- Botox paralyzes skeletal muscle but clearly has some impact on smooth muscle — Marc Levitt (clinical) [Ep 15 · 1:57](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=117)
- Babies have very tight anal sphincter with or without Hirschsprung's disease, and if they hold stool in successfully, normal babies get constipated while Hirschsprung babies get enterocolitis — Marc Levitt (clinical) [Ep 15 · 2:06](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=126)
- After a perfectly done operation that preserves 1 centimeter of anal canal without hurting sphincters, high tone will occur in a baby that doesn't know how to relax — Marc Levitt (clinical) [Ep 15 · 2:26](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=146)
- Botox is valuable for babies coming back with enterocolitis episodes after ensuring no anatomic or pathologic problem with the pull-through — Marc Levitt (clinical) [Ep 15 · 3:15](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=195)
- In the early period up to one year of age, bad behavior by the patient can occur even with a perfectly done pull-through — Marc Levitt (clinical) [Ep 15 · 3:27](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=207)
- After one year of age, there may be an anatomic or pathologic problem, and Botox is not very valuable because the underlying problem must be identified — Marc Levitt (clinical) [Ep 15 · 3:36](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=216)
- A retained cuff that is too big, not split completely, or rolled up is particularly offensive to the ability of the pull-through to empty — Marc Levitt (clinical) [Ep 15 · 4:47](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=287)
- Patients with retained cuff will get better temporarily with Botox but will continue to fail every 2 or 3 months — Marc Levitt (clinical) [Ep 15 · 5:09](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=309)
- Nerves greater than 40 microns indicate a transition zone pull-through — Marc Levitt (clinical) [Ep 15 · 5:35](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=335)
- 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 (opinion) [Ep 15 · 5:43](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=343)
- When anatomic and pathologic issues are completely ruled out (no twist, stricture, cuff, etc.), Botox may be done once or maybe twice and then the patient is done — Marc Levitt (clinical) [Ep 15 · 5:58](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=358)
- If a patient over 1 year of age keeps misbehaving, there is likely an anatomic or pathologic problem — Marc Levitt (clinical) [Ep 15 · 6:14](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=374)
- Dr. Levitt has never met a patient with Hirschsprung disease that is anatomically perfect (no stricture, cuff, duhamel pouch causing trouble, twist) with normal ganglion cells and nerves less than 40 microns who does not spontaneously empty, except rare patients under one year who have not learned to relax their anal canal — Marc Levitt (opinion) [Ep 15 · 6:24](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=384)
- If symptoms persist in an older child, there is an anatomic or pathologic problem that has not yet been identified — Marc Levitt (clinical) [Ep 15 · 7:00](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=420)
- Dr. Levitt's Botox technique: 100 units in 10 cc saline, injecting 2.5 cc submucosal into each quadrant with a very small gauge needle — Marc Levitt (clinical) [Ep 15 · 7:07](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=427)
- Dr. Levitt will never do internal sphincterotomy because it is permanent Botox and could cause permanent incontinence — Marc Levitt (opinion) [Ep 15 · 7:30](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=450)
- Most practitioners use between 60 and 100 units of Botox for Hirschsprung patients — Scott Ingham (clinical) [Ep 15 · 8:08](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=488)
- Some practitioners use much less volume (1 mL total with 0.25 mL per quadrant) compared to Dr. Levitt's 10 mL technique — Scott Ingham (clinical) [Ep 15 · 8:44](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=524)
- A dilated pull-through segment may be secondary to noncompliance with dilations or bowel regimen, or the surgeon may not have taken out enough bowel at the original surgery — Marc Levitt (clinical) [Ep 15 · 9:35](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=575)
- Patients with dilated segment and no other anatomic abnormality who continue to misbehave may be offered redo surgery to remove the dilated segment, though this is exceedingly rare — Marc Levitt (clinical) [Ep 15 · 10:01](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=601)
- Almost always there is something causing the dilation, either a cuff or a transition zone segment of bowel — Marc Levitt (clinical) [Ep 15 · 10:13](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=613)
- The workup for patients with dilated segments includes contrast study, examination under anesthesia, and rectal biopsy — Marc Levitt (clinical) [Ep 15 · 10:34](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=634)
- For redo pull-through, Dr. Levitt performs transanal approach saving the anal canal, delivers bowel into abdomen, then decides whether tapering is needed — Marc Levitt (clinical) [Ep 15 · 11:11](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=671)
- A tapered segment will be fairly dysmotile for many months — Marc Levitt (clinical) [Ep 15 · 11:24](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=684)
- Dr. Levitt always diverts patients with an ileostomy if tapering or redo is required — Marc Levitt (clinical) [Ep 15 · 11:31](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=691)
- Most likely the dilated segment can be removed and healthy segment brought down without tapering — Marc Levitt (clinical) [Ep 15 · 11:37](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=697)
- It is critical to ensure removal of any distal obstruction during redo surgery; Dr. Levitt has seen patients redone with Soave cuff left alone, requiring redo of the redo to remove the cuff before improvement — Marc Levitt (clinical) [Ep 15 · 11:46](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-audience-q-a-with-dr-marc-levitt-3440?t=706)
- Hirschsprung disease occurs in about 1 in 5000 live births. — Marc Levitt (epidemiological) [Ep 18 · 3:30](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=210)
- In Hirschsprung disease, ganglion cells have not migrated to the distal colon, and without ganglion cells the colon cannot relax and therefore stays squeezed. — Marc Levitt (clinical) [Ep 18 · 4:53](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=293)
- 90% of Hirschsprung patients are diagnosed in the first couple of months of life, the vast majority in the first week or so of life. — Marc Levitt (epidemiological) [Ep 18 · 4:42](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=282)
- Because the lining of the bowel is not normal in Hirschsprung disease, bacteria that stay in the colon can migrate out and get into the bloodstream, creating a life-threatening condition called enterocolitis. — Marc Levitt (clinical) [Ep 18 · 6:05](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=365)
- If Hirschsprung disease is recognized, it is pretty straightforward to intervene; you do not necessarily need surgery to prevent enterocolitis, you just need good nursing care and proper irrigation to get the stool to flow. — Marc Levitt (clinical) [Ep 18 · 7:06](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=426)
- About 5% of Hirschsprung patients present after 1 year of life; the vast majority present as babies. — Marc Levitt (epidemiological) [Ep 18 · 8:10](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=490)
- Down syndrome is associated with Hirschsprung disease. — Marc Levitt (clinical) [Ep 18 · 9:14](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=554)
- 10% of patients with meconium plug actually have Hirschsprung disease as the underlying cause; 90% just pass the plug and get better. — Marc Levitt (epidemiological) [Ep 18 · 10:46](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=646)
- Milk protein allergy can mimic Hirschsprung disease; if you biopsy looking for Hirschsprung, you will find ganglion cells but lots of eosinophils. — Marc Levitt (clinical) [Ep 18 · 11:22](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=682)
- To confirm Hirschsprung disease, you need both the absence of ganglion cells and confirmation that the nerves associated with those ganglion cells are thickened (hypertrophic). — Marc Levitt (clinical) [Ep 18 · 13:40](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=820)
- Nerve trunks greater than 40 microns are abnormal in rectal biopsy specimens. — Marc Levitt (clinical) [Ep 18 · 14:31](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=871)
- You cannot diagnose Hirschsprung with a frozen section, but you can rule it out if ganglion cells are present. To prove it is Hirschsprung disease, you need several days and need to evaluate 100 slices of the sample. — Marc Levitt (clinical) [Ep 18 · 16:37](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=997)
- In about 15% of Hirschsprung cases, the aganglionic segment extends higher than the splenic flexure. — Marc Levitt (epidemiological) [Ep 18 · 20:41](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1241)
- 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 (clinical) [Ep 18 · 22:09](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1329)
- The dilated colon in Hirschsprung disease will shrink down to more normal size as soon as it is given the opportunity to successfully empty out the anus once the blockade has been removed. — Marc Levitt (clinical) [Ep 18 · 22:43](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1363)
- You really only need about 10% of your colon to function completely normally and have one bowel movement per day. — Marc Levitt (clinical) [Ep 18 · 29:06](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1746)
- Most Hirschsprung patients only lose about 15 to 20% of their colon because that is where the abnormal segment is, so they can have a completely normal stooling life with one bowel movement per day. — Marc Levitt (clinical) [Ep 18 · 29:14](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1754)
- Patients who have to lose their entire colon and have small bowel brought to the anus tend to have more frequent stools, somewhere between 2 and 6 per day, but can all maintain bowel control provided the surgeon successfully preserves the anal canal and sphincters. — Marc Levitt (clinical) [Ep 18 · 29:29](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1769)
- Sometimes the irrigation tube does not reach high enough and does not get into the normal bowel, and therefore you cannot successfully decompress the bowel; such patients would benefit from a diversion with a stoma. — Marc Levitt (clinical) [Ep 18 · 30:46](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=1846)
- In Hirschsprung disease, the internal sphincter does not relax normally. When you have fullness in your rectum, your internal sphincter is supposed to relax, but in Hirschsprung disease it tightens at the wrong time and can hold stool in at an inappropriate time. — Marc Levitt (clinical) [Ep 18 · 42:39](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2559)
- Even after successful Hirschsprung surgery, you are in danger of getting enterocolitis if you do not have good flow, and the sphincters can slow down the flow enough that you can develop enterocolitis even after surgery. — Marc Levitt (clinical) [Ep 18 · 41:43](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2503)
- If you give Botox to temporarily knock out the sphincters (the Botox wears off over the next 3 months), the baby can learn to push on their abdomen and overcome the non-relaxing sphincters. — Marc Levitt (clinical) [Ep 18 · 41:59](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2519)
- The vast majority of Hirschsprung patients recover very uneventfully, stool normally, and when they get to age 3 or 4 they successfully potty train. — Marc Levitt (clinical) [Ep 18 · 43:59](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2639)
- Hirschsprung disease is a source of significant morbidity and mortality in the developing world if it is unrecognized. — Marc Levitt (epidemiological) [Ep 18 · 44:52](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2692)
- If a family has a baby with Hirschsprung disease, the risk of another baby having Hirschsprung disease in their family is about 1 in 200, significantly higher than the general population risk of 1 in 5000. — Marc Levitt (epidemiological) [Ep 18 · 38:40](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2320)
- Hirschsprung-associated enterocolitis is treated with irrigations, metronidazole antibiotic (for anaerobic bacteria), and intravenous hydration. — Marc Levitt (clinical) [Ep 18 · 39:29](https://qa.library.globalcastmd.com/watch/drbeen-medical-lectures-dr-marc-levitt-md-discusses-hirschsprung-disease-6227?t=2369)
- Trisomy 21 is associated with approximately 50 times higher incidence of Hirschsprung disease compared to non-trisomy 21 patients, with about 5-10% of trisomy 21 patients having Hirschsprung disease. — Lily Cheng (epidemiological) [Ep 6 · 2:58](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=178)
- Hirschsprung disease is almost never an emergency operation, and irrigations usually win the day. — Marc Levitt (clinical) [Ep 6 · 9:52](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=592)
- In Hirschsprung disease with perforation, the cecum perforates due to Laplace's law, and this indicates the transition zone is probably around the hepatic flexure because the right colon becomes very dilated with nowhere to empty. — Marc Levitt (clinical) [Ep 6 · 5:52](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=352)
- In anorectal malformation patients with perforation, the sigmoid colon typically perforates with a linear, longitudinal tear along the taenia. — Jason Frischer (clinical) [Ep 6 · 6:27](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=387)
- The pathophysiology of Hirschsprung enterocolitis involves physiologic obstruction at both the sphincter level and in the aganglionic segment, leading to stasis, bacterial overgrowth, translocation, and sepsis. — Jason Frischer (clinical) [Ep 6 · 7:34](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=454)
- Proper irrigation technique involves instilling small aliquots of warm saline (20-40 mL at a time) and actively withdrawing with a catheter to evacuate the saline, not just infusing it into the colon lumen. — Marc Levitt (clinical) [Ep 6 · 8:11](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=491)
- Cold saline used for irrigations in small children can significantly change the child's body temperature, so warm saline should be used. — Jason Frischer (clinical) [Ep 6 · 8:41](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=521)
- The rectosigmoid ratio less than one on contrast enema is indicative of Hirschsprung disease. — Lily Cheng (clinical) [Ep 6 · 10:40](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=640)
- Sawtoothing in the rectum on contrast enema represents hyperperistalsis and is very classic for Hirschsprung disease, particularly when associated with enterocolitis. — Jason Frischer (clinical) [Ep 6 · 10:55](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=655)
- Definitive pathologic diagnosis of Hirschsprung disease requires absence of ganglion cells in 100 levels and presence of hypertrophic nerves greater than 40 microns. — Marc Levitt (clinical) [Ep 6 · 11:28](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=688)
- Frozen section can only definitively tell you it is NOT Hirschsprung disease (if ganglion cells are present), but cannot definitively confirm it IS Hirschsprung disease because that requires 100 levels with no ganglion cells anywhere. — Marc Levitt (clinical) [Ep 6 · 11:28](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=688)
- Calretinin staining is used as an adjunct: if calretinin is present, ganglion cells are nearby; if calretinin stain is absent, this further confirms Hirschsprung disease. — Christy Raylan (clinical) [Ep 6 · 11:54](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=714)
- 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 (opinion) [Ep 6 · 13:33](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=813)
- Literature from the PCPLC and Michael Rollins shows that delayed definitive surgery for Hirschsprung disease (around 3 months out) has similar outcomes, so it is okay to wait as long as patient receives good irrigations and is growing and healthy. — Jason Frischer (clinical) [Ep 6 · 14:29](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=869)
- If a baby with Hirschsprung disease is improving with irrigations but cannot be fed, diversion is a reasonable next step to allow enteral nutrition and growth. — Marc Levitt (clinical) [Ep 6 · 15:20](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=920)
- For leveling biopsies, the optimal strategy is to go directly to the sigmoid ('go for the money') and if frozen section shows ganglion cells, no other biopsies are necessary. — Marc Levitt (clinical) [Ep 6 · 16:14](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=974)
- If mapping the colon without frozen section available, the entire colon should be mapped (left colon, transverse colon, and hepatic flexure/right colon) to avoid missing ganglionic segments. — Jason Frischer (clinical) [Ep 6 · 17:23](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1043)
- The appendix should not be biopsied for Hirschsprung mapping because it is not helpful and should be saved for potential future use; many patients' appendixes are aganglionic. — Marc Levitt (clinical) [Ep 6 · 18:06](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1086)
- Frozen section interpretation can be difficult in the setting of active enterocolitis due to excessive inflammation obscuring ganglion cells. — Christy Raylan (clinical) [Ep 6 · 20:34](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1234)
- In resource-limited settings without frozen section availability, the strategy is to bring up the dilated portion of colon as ostomy because it is more likely to be functional. — Marc Levitt (clinical) [Ep 6 · 21:01](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1261)
- In resource-limited settings, ileostomy is not a good option because patients cannot access medical care quickly enough if they become dehydrated, so colostomy is preferred. — Marc Levitt (clinical) [Ep 6 · 21:01](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1261)
- Modern telemedicine technology allows pathology slides (H&E stains) to be photographed through microscope and sent via internet for remote ganglion cell evaluation, helping save colons in resource-limited settings. — Marc Levitt (clinical) [Ep 6 · 21:35](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1295)
- By pure numbers, 80% of Hirschsprung disease cases are rectosigmoid, so remote pathology evaluation saves many colons. — Marc Levitt (epidemiological) [Ep 6 · 21:35](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1295)
- In settings where patients can be kept well hydrated with easy healthcare access, ileostomy is preferred over colostomy for diversion because colostomy mesentery becomes shortened and inflamed after division, making subsequent pull-through technically difficult with inadequate reach. — Jason Frischer (clinical) [Ep 6 · 22:52](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1372)
- When performing ileostomy for Hirschsprung diversion, frozen section should be done on the ileostomy to ensure it will function. — Jason Frischer (clinical) [Ep 6 · 23:41](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1421)
- If a surgeon does a good job with pull-through using elegant technique, preserving the anal canal, and not overstretching sphincters, the patient will still get some enterocolitis because the continence mechanism is preserved and patients cannot relax their internal sphincter. — Marc Levitt (clinical) [Ep 6 · 24:15](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1455)
- Patients whose sphincters were overstretched during Hirschsprung surgery developed fecal incontinence but never got enterocolitis, demonstrating the relationship between sphincter function and enterocolitis risk. — Marc Levitt (clinical) [Ep 6 · 24:15](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1455)
- A study of Botox injection at one month post-operatively for Hirschsprung disease showed it did not help prevent enterocolitis (negative study published). — Marc Levitt (clinical) [Ep 6 · 26:10](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1570)
- Cincinnati group is conducting a non-randomized study of Botox injection at the anal sphincter at time of ileostomy closure, with retrospective baseline comparison and prospective data collection currently in mid-30s patients; some patients in the protocol have still developed enterocolitis, so it is not 100% effective. — Jason Frischer (clinical) [Ep 6 · 26:54](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1614)
- Families should be sent home with equipment to irrigate and taught how to irrigate before the pull-through so they can practice, ensuring they know the technique before discharge and avoiding catheter passage through fresh anastomosis post-op day 5. — Jason Frischer (clinical) [Ep 6 · 28:04](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1684)
- Harold Hirschsprung identified that a baby could be sick due to this problem but did not understand the pathology. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- The disease is named Hirschsprung disease, not apostrophe S. — Marc Levitt (guideline) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Orvar Swenson figured out the pathology by going to the pathology lab and defined the fact that there were no ganglion cells. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Prior to Swenson's work, removal of the dilated colon was the treatment, which was a mistake; it was the distal narrow colon that was the problem. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Swenson developed the first operation for Hirschsprung disease, a full-thickness rectal dissection. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Some surgeons still do Suave procedures, but they are becoming more Swenson-like with maybe a one-centimeter cuff; Dan von Allman calls those 'Suavesons with a one-centimeter cuff.' — Marc Levitt (opinion) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Dr. Yancey was the first surgeon to describe a submucosal dissection for Hirschsprung disease, but published in a journal not widely read; Dr. Suave published later in a more widely read journal, so the technique is called Suave rather than Yancey. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- The Suave technique was developed because people said the Swenson caused fecal and urinary incontinence or voiding dysfunction; Swenson himself wrote that the operation was good but surgeons were doing it wrong by dissecting too wide. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Doing a proper Swenson right on the bowel wall, like a PSARP right on the bowel wall, avoids nerve injury; if you see fat you can get closer, as the nerves are in the fatty layer. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Swenson was 105 when he died and used to write letters to Levitt and Alberto Pena asking them to promote the Swenson operation. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Duhamel had the idea to leave the original rectum behind and do a pull-through next to it, then mate the two lumens. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- The Duhamel is now really only appropriate for an ilio-Duhamel, although Levitt would still do an ilioanal. — Marc Levitt (opinion) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Rabine did a low anterior resection for Hirschsprung disease, leaving about six centimeters behind; some patients did fine as ganglionated bowel pooped through the six centimeters of aganglionated bowel, but that operation has gone to the wayside. — Marc Levitt (clinical) [Ep 17 · 0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26)
- Dr. Boley was the first to do the primary coloanal anastomosis of a Suave, eliminating the need to leave the bowel hanging out and come back at day seven; the proper description is the Suave technique with the Boley modification, i.e., a Suave-Boley. — Marc Levitt (clinical) [Ep 17 · 5:57](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=357)
- Henry So was a pediatric surgeon in the Philippines and the first to do a primary pull-through (transabdominal) with no preceding stoma, because patients with stomas at home in the Philippines faced such social stigma that babies were basically left to die by their families. — Marc Levitt (clinical) [Ep 17 · 5:57](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=357)
- Dr. Martin was the first surgeon-in-chief and pediatric surgeon at Cincinnati Children's Hospital. — Jason Frischer (clinical) [Ep 17 · 9:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=580)
- Dr. Martin developed the Martin procedure, an expansion of the Duhamel for long-segment Hirschsprung disease, leaving a longer aganglionated segment of rectum and pulling through ganglionated bowel. — Jason Frischer (clinical) [Ep 17 · 9:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=580)
- Dr. Martin's biggest contribution was in ulcerative colitis; in 1977, before the J-pouch, he took the endorectal pull-through technique used in Hirschsprung disease and applied it to ulcerative colitis, doing a total proctocolectomy with ilioanal anastomosis. — Marc Levitt (clinical) [Ep 17 · 10:34](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=634)
- The transanal dissection and Suave plane dissection is the same concept as the mucosectomy in ulcerative colitis. — Marc Levitt (clinical) [Ep 17 · 10:34](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=634)
- Helen Noblet figured out the suction rectal biopsy; she is from Melbourne, Australia. — Marc Levitt (clinical) [Ep 17 · 11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701)
- Keith Jorgensen did the laparoscopic version of the Suave; in his original description (with Tom Inge on the paper), they talked about leaving a five-centimeter cuff, which nowadays would be way too much. — Marc Levitt (clinical) [Ep 17 · 11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701)
- Jack Langer approached Hirschsprung disease transanally, doing a transanal resection of the rectosigmoid with or without laparoscopy or laparotomy. — Marc Levitt (clinical) [Ep 17 · 11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701)
- Luis de la Torre also did transanal resection around the same time as Jack Langer; some places around the world are doing transanal only, and Levitt does that in certain circumstances. — Marc Levitt (clinical) [Ep 17 · 11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701)
- Dan Teitelbaum did an incredible amount of work in Hirschsprung disease, particularly significant research in enterocolitis; he passed away from a brain tumor. — Marc Levitt (clinical) [Ep 17 · 11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701)
- Laparoscopic approach allows ability to biopsy elsewhere if transition zone is not rectosigmoid — Andrea Badillo (clinical) [Ep 4 · 5:43](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=343)
- Deep laparoscopic dissection into pelvis minimizes transanal work needed and avoids sphincter overstretching morbidity — Andrea Badillo (clinical) [Ep 4 · 6:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=360)
- Transanal dissection should take under one hour in primary pull-through — Marc Levitt (clinical) [Ep 4 · 6:36](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=396)
- Full-thickness biopsy must include seromuscular side same size as mucosal side (cube-shaped) — Aaron Garrison (clinical) [Ep 4 · 7:09](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=429)
- Should wait for frozen section before taking mesentery — Andrea Badillo (clinical) [Ep 4 · 9:30](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=570)
- Adequate frozen section requires ganglion cells and nerves less than 40 microns — Andrea Badillo (clinical) [Ep 4 · 10:08](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=608)
- Frozen section biopsy must include submucosa to avoid missing hypertrophic nerves in submucosal layer while finding ganglion cells in seromuscular layer — Jason Frischer (clinical) [Ep 4 · 10:19](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=619)
- Mesenteric dissection should stay close to bowel wall, not deep in mesentery, to reduce bleeding — Jason Frischer (clinical) [Ep 4 · 11:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=660)
- In old Swenson procedures done through abdomen, too wide distal rectal dissection caused incontinence and urinary retention from nerve injury — Marc Levitt (clinical) [Ep 4 · 11:42](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=702)
- Must preserve arcade along left colon and sigmoid to get enough length to reach pelvis — Jason Frischer (clinical) [Ep 4 · 12:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=746)
- For distal disease, can take just distal IMA branches; for left colon disease may need to take IMA to get splenic flexure to reach — Marc Levitt (clinical) [Ep 4 · 12:35](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=755)
- Transanal dissection should start about one centimeter above dentate line — Andrea Badillo (clinical) [Ep 4 · 13:02](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=782)
- Swenson full-thickness dissection follows areolar plane which is essentially bloodless — Marc Levitt (clinical) [Ep 4 · 14:12](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=852)
- Soave submucosal dissection leaves a cuff; if performed, cuff should be very short (about one centimeter) and must be split — Marc Levitt (clinical) [Ep 4 · 14:12](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=852)
- Resection margin should be approximately five centimeters above biopsy site where bowel looks good — Jason Frischer (clinical) [Ep 4 · 16:06](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=966)
- Tacking sutures from serosa to pelvic sidewall at three and six o'clock positions anchor anastomosis in place — Aaron Garrison (clinical) [Ep 4 · 16:30](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=990)
- Harold Hirschsprung figured out that a baby could be sick due to this problem but did not understand the pathology — Marc Levitt (clinical) [Ep 16 · 1:03](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=63)
- The correct name is Hirschsprung disease without apostrophe S — Marc Levitt (guideline) [Ep 16 · 1:16](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=76)
- Orvar Swenson figured out the pathology by going to the pathology lab and defined the fact that there were no ganglion cells — Marc Levitt (clinical) [Ep 16 · 1:24](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=84)
- Prior to Swenson's work, removal of the dilated colon was the treatment, which was a mistake because it was the distal narrow colon that was the problem — Marc Levitt (clinical) [Ep 16 · 1:35](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=95)
- Swenson developed the first operation for Hirschsprung disease, which is a full-thickness rectal dissection — Marc Levitt (clinical) [Ep 16 · 1:46](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=106)
- Modern Suave procedures are becoming more Swenson-like by making only a 1 centimeter cuff, which Dan von Almen describes as basically Swensons with a 1 centimeter cuff — Marc Levitt (clinical) [Ep 16 · 2:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=120)
- The Suave procedure should properly be called the Yancey because Doctor Yancey first described submucosal dissection for Hirschsprung disease but published in a journal not widely read, while Suave published years later in a more widely read journal — Marc Levitt (clinical) [Ep 16 · 2:13](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=133)
- The Suave/Yancey technique was developed because people said the Swenson caused fecal and urinary incontinence or voiding dysfunction after the operation — Marc Levitt (clinical) [Ep 16 · 2:42](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=162)
- Doctor Swenson wrote a paper saying the complications were due to surgeons doing the operation wrong by dissecting too wide, not from the operation itself — Marc Levitt (clinical) [Ep 16 · 2:56](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=176)
- Yancey, Suave, and Duhamel did everything in their power to avoid full-thickness rectal dissection and stay out of the rectal plane to avoid injury to the nerve erigentes — Marc Levitt (clinical) [Ep 16 · 3:10](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=190)
- A proper Swenson done right on the bowel wall avoids nerve injury because the nerves are in the fatty layer; dissecting too wide will injure them — Marc Levitt (clinical) [Ep 16 · 3:30](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=210)
- Orvar Swenson died at age 105 and wrote letters to Mark Levitt and Alberto Pena asking them to tell everybody the Swenson is a good operation — Marc Levitt (clinical) [Ep 16 · 3:48](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=228)
- Duhamel had the idea to leave the original rectum behind and do a pull-through next to it, then mate the two lumens — Marc Levitt (clinical) [Ep 16 · 4:06](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=246)
- The Duhamel is now only appropriate for an ileoduhamel, though the speaker would still do an ileoanal — Marc Levitt (opinion) [Ep 16 · 4:22](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=262)
- Rabine did a low anterior resection for Hirschsprung leaving about 6 centimeters of aganglionic bowel behind, and some patients did perfectly fine with ganglionated bowel pooping through the aganglionic segment — Marc Levitt (clinical) [Ep 16 · 4:35](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=275)
- Doctor Boley was the first to do primary coloanal anastomosis of a Suave, eliminating the need to leave bowel hanging out and return at day 7 — Marc Levitt (clinical) [Ep 16 · 6:15](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=375)
- The proper description is the Suave technique with the Boley modification, abbreviated as Suave-Boley — Marc Levitt (guideline) [Ep 16 · 6:36](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=396)
- Hung Bae Kim figured out the STEP procedure — Marc Levitt (clinical) [Ep 16 · 8:23](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=503)
- Doctor Henrys in the Philippines was the first surgeon to do a primary pull-through with no preceding stoma — Marc Levitt (clinical) [Ep 16 · 8:39](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=519)
- Henrys performed primary pull-throughs because patients with stomas at home in the Philippines were not cared for due to social stigma, and babies were basically left to die by their families — Marc Levitt (clinical) [Ep 16 · 8:58](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=538)
- Doctor Martin developed the Martin procedure, an expansion of the Duhamel leaving a longer aganglionic segment of rectum and pulling through ganglionated bowel for long-segment Hirschsprung disease — Jason Frischer (clinical) [Ep 16 · 10:09](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=609)
- Doctor Martin published in 1977 the first description of applying endorectal pull-through techniques from Hirschsprung surgery to ulcerative colitis treatment, performing total proctocolectomy with ileoanal anastomosis using transanal dissection and Suave plane dissection — Jason Frischer (clinical) [Ep 16 · 10:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=640)
- Helen Noblet from Melbourne, Australia figured out the suction rectal biopsy — Marc Levitt (clinical) [Ep 16 · 12:32](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=752)
- Keith Jorgeson did the laparoscopic version of the Suave — Marc Levitt (clinical) [Ep 16 · 12:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=760)
- In Jorgeson's original description with Tom Hinge, they talked about leaving a 5 centimeter cuff, which nowadays would be way too much — Marc Levitt (clinical) [Ep 16 · 12:57](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=777)
- Jack Langer and Luis de la Torre developed transanal resection of the rectosigmoid with or without laparoscopy or laparotomy — Marc Levitt (clinical) [Ep 16 · 13:17](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=797)
- Some places around the world are doing transanal-only approaches for Hirschsprung disease — Marc Levitt (clinical) [Ep 16 · 13:43](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=823)
- Dan Teitelbaum did a significant amount of research in enterocolitis related to Hirschsprung disease — Marc Levitt (clinical) [Ep 16 · 13:51](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=831)
- Late-onset Hirschsprung patients tend to present with more subtle symptoms, more constipation, and fewer issues with enterocolitis compared to neonatal presentations. — Marc Levitt (clinical) [Ep 10 · 3:21](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=201)
- Frozen section pathology for Hirschsprung can be misleading; marking biopsy sites with prolene stitches and obtaining permanent pathology before pull-through is preferable. — Martin Latcher (clinical) [Ep 10 · 4:08](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=248)
- If biopsy sites are not marked, it is very hard to find them in the future. — Marc Levitt (clinical) [Ep 10 · 4:20](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=260)
- The laparoscopic approach allows preservation of the colonic arcade and the inferior mesenteric artery without overstretching the anus, resulting in a well-perfused, tension-free pull-through. — Martin Latcher (clinical) [Ep 10 · 4:51](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=291)
- The laparoscopic portion of Hirschsprung pull-through minimizes the amount of anal stretch required and is very precise and elegant. — Marc Levitt (opinion) [Ep 10 · 5:06](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=306)
- In older children with Hirschsprung where the transition zone level is confirmed, transanal-only approach may be considered because you know exactly where the transition zone is and can reach it. — Marc Levitt (clinical) [Ep 10 · 5:21](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=321)
- A disadvantage of taking biopsies at the time of ileostomy creation is that adhesions often form between former biopsy sites and the lateral abdominal wall. — Martin Latcher (clinical) [Ep 10 · 7:23](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=443)
- The anastomotic donut should be divided into four quadrants to ensure ganglion cells are present circumferentially, because the transition zone is sometimes like a lip and not circumferential. — Martin Latcher (clinical) [Ep 10 · 7:53](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=473)
- The commonly stated rule to go 5 centimeters above the transition zone is actually not true; there is often a longer transition zone in many cases. — Marc Levitt (clinical) [Ep 10 · 11:14](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=674)
- There are advantages to having the ultimate pull-through nice and straight (usually upper sigmoid) if you ever need to irrigate. — Marc Levitt (clinical) [Ep 10 · 11:28](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=688)
- It is very important to send a full-thickness biopsy cube, not a diamond; the seromuscular layer square needs to be the same size as the mucosa square to prevent errors from tangential cutting if the specimen is not oriented correctly. — Marc Levitt (clinical) [Ep 10 · 11:49](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=709)
- Ganglion cell distribution should be visualized like a paint can with ganglion cells dripping down the sides, not as concentric circles; you could theoretically biopsy in a transition zone where there is only a drip of paint, not where the paint is absent. — Marc Levitt (clinical) [Ep 10 · 12:18](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=738)
- Pathologists doing Hirschsprung biopsies correctly will refuse to do analysis unless you give them submucosa, because checking seromuscular layer only is a cardinal sin. — Marc Levitt (clinical) [Ep 10 · 13:17](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=797)
- The pathologist must confirm that not only is the seromuscular layer good, but the submucosal layer is also good, because seromuscular could have ganglion cells while submucosal layer could have hypertrophic nerves. — Marc Levitt (clinical) [Ep 10 · 13:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=817)
- The results of reoperative transanal Hirschsprung pull-throughs are not great, with a high number of anastomotic dehiscences, partly because you can never enter the anal canal in the same plane during a redo. — Martin Latcher (clinical) [Ep 10 · 14:16](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=856)
- If you go back early after initial pull-through, you can release the stitches and arrive in the same plane again, allowing re-anastomosis to the anal canal exactly in the same plane as the initial surgery, which is why early re-operation avoids dehiscence. — Martin Latcher (clinical) [Ep 10 · 14:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=877)
- Reoperative transanal Hirschsprung surgery is not as complicated if you go back early. — Martin Latcher (opinion) [Ep 10 · 15:07](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=907)
- Trisomy 21 is associated with approximately 50 times higher incidence of Hirschsprung disease compared to non-trisomy 21 patients, with about 5-10% of trisomy 21 patients having Hirschsprung disease. — Lily Cheng (epidemiological) [Ep 5 · 3:01](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=181)
- Hirschsprung disease is almost never an emergency operation; irrigation usually wins the day. — Marc Levitt (clinical) [Ep 5 · 9:54](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=594)
- Rectal irrigations for Hirschsprung enterocolitis should use 20 mL per kg, 3 times a day, with warm saline to avoid hypothermia in small children. — Lily Cheng (clinical) [Ep 5 · 4:11](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=251)
- Irrigation technique requires instilling small aliquots of saline (20-40 mL at a time) and then evacuating it rather than just infusing into the colon lumen. — Jason Frischer (clinical) [Ep 5 · 8:11](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=491)
- In Hirschsprung disease with free air, perforation typically occurs in the cecum when the transition zone is around the hepatic flexure, due to Laplace's law causing the cecum to perforate as the most dilated segment. — Marc Levitt (clinical) [Ep 5 · 5:49](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=349)
- In anorectal malformation patients with delayed diagnosis, perforation typically occurs in the sigmoid colon as a linear longitudinal tear along the taenia. — Marc Levitt (clinical) [Ep 5 · 6:27](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=387)
- Definitive pathologic diagnosis of Hirschsprung disease requires absence of ganglion cells on 100 levels and presence of hypertrophic nerves greater than 40 microns. — Marc Levitt (clinical) [Ep 5 · 11:28](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=688)
- Frozen section can only definitively tell you it is NOT Hirschsprung disease by showing ganglion cells, but cannot confirm it IS Hirschsprung disease because that requires 100 levels showing no ganglion cells anywhere. — Marc Levitt (clinical) [Ep 5 · 11:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=697)
- Calretinin staining is used as an adjunct diagnostic tool; if calretinin is present it indicates ganglion cells are nearby, while absent calretinin further confirms Hirschsprung disease. — Marc Levitt (clinical) [Ep 5 · 11:54](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=714)
- Experts recommend waiting minimum 4 weeks after treating enterocolitis before performing definitive pull-through surgery, as long as the patient is receiving good irrigations and growing and healthy otherwise. — Jason Frischer (clinical) [Ep 5 · 14:29](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=869)
- Literature from the PCPLC and Michael Rawlins shows that delayed definitive surgery for Hirschsprung disease (around 3 months out) has similar outcomes to earlier surgery. — Jason Frischer (clinical) [Ep 5 · 14:39](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=879)
- If a Hirschsprung patient is not being fed due to ongoing issues, diversion is a reasonable next step rather than continuing irrigations at home. — Marc Levitt (clinical) [Ep 5 · 15:08](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=908)
- For leveling biopsies, the recommended strategy is to start with sigmoid biopsy; if frozen section shows ganglion cells, no other biopsies are necessary. — Marc Levitt (clinical) [Ep 5 · 16:14](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=974)
- When mapping the colon without frozen section available, biopsies should be taken from left colon, transverse colon, and hepatic flexure/distal ascending colon, as cecal pull-throughs do not succeed very well. — Jason Frischer (clinical) [Ep 5 · 17:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1057)
- The appendix should not be biopsied for Hirschsprung mapping because many patients' appendixes are aganglionic and it should be saved for possible future use. — Marc Levitt (clinical) [Ep 5 · 18:26](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1106)
- When going to OR electively for pull-through and finding no ganglion cells in sigmoid/left colon, the surgeon should not proceed with pull-through that day but should biopsy transverse colon and hepatic flexure, do an ileostomy, and wait for permanent section before discarding any colon. — Marc Levitt (clinical) [Ep 5 · 19:32](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1172)
- Ileostomy is preferred over colostomy for diversion in Hirschsprung disease because colonic diversion can shorten the mesentery through division and inflammation, making subsequent pull-through technically more difficult with inadequate reach. — Jason Frischer (clinical) [Ep 5 · 23:05](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1385)
- Frozen section should be performed on the ileostomy site to ensure it will function properly. — Marc Levitt (clinical) [Ep 5 · 23:48](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1428)
- Frozen section interpretation can be unreliable in the setting of active enterocolitis due to inflammation obscuring ganglion cells. — Christy Ryan (clinical) [Ep 5 · 20:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1237)
- In resource-limited settings without frozen section capability, the strategy is to bring up the dilated portion of colon which is more likely to be functional, and ileostomy is not an option due to inability to manage dehydration with quick medical access. — Marc Levitt (clinical) [Ep 5 · 21:04](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1264)
- Modern telemedicine technology allows pathologists to evaluate H&E stained slides for ganglion cells via Zoom using a camera and microscope, enabling better surgical planning in resource-limited settings and potentially saving 80% of colons in rectosigmoid Hirschsprung disease. — Jason Frischer (clinical) [Ep 5 · 21:41](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1301)
- Patients who had Hirschsprung surgery with overstretched sphincters developed fecal incontinence but never got enterocolitis, demonstrating that sphincter preservation paradoxically maintains susceptibility to enterocolitis. — Marc Levitt (clinical) [Ep 5 · 24:31](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1471)
- Hirschsprung patients cannot relax their internal sphincter even after pull-through, which is why they remain susceptible to enterocolitis despite technically successful surgery. — Marc Levitt (clinical) [Ep 5 · 25:14](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1514)
- A published study of Botox injection at one month post-operatively for Hirschsprung disease showed no benefit in preventing enterocolitis. — Marc Levitt (clinical) [Ep 5 · 26:04](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1564)
- Botox injection at the anal sphincter at time of ileostomy closure is being studied prospectively but is not 100% effective, as some patients in the protocol still developed enterocolitis. — Jason Frischer (clinical) [Ep 5 · 27:05](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1625)
- Families should be sent home with irrigation equipment and taught irrigation technique before pull-through surgery, with parents practicing the technique, to avoid having catheters go through the anastomosis post-operatively when issues arise. — Jason Frischer (clinical) [Ep 5 · 28:04](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1684)
- Contrast enema findings of rectosigmoid ratio less than 1, saw-toothing pattern in the rectum, and hyperperistalsis are indicative of Hirschsprung disease with associated enterocolitis. — Lily Cheng (clinical) [Ep 5 · 10:40](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=640)
- Approximately 80% of Hirschsprung disease cases are rectosigmoid in extent. — Jason Frischer (epidemiological) [Ep 5 · 22:03](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1323)

## Host summaries
Recaps by a host of what the experts said — not the host's own clinical position.
- The negative appendectomy rate among hospitals is a median of 2.6% with a range from 0 to 17%, according to Kurt Newman's 2003 paper in Journal of Pediatric Surgery reviewing the PHIS database. — Nick Bruns summarizing the discussion [Ep 3 · 1:59](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=119)
- For incidental Meckel's diverticulum during laparoscopy, Zani's 2008 Annals of Surgery review recommended leaving it, stating the risk of complications is not worth the extremely low mortality rate of 0.001%. — Nick Bruns summarizing the discussion [Ep 3 · 2:44](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=164)
- The 50-year Mayo Clinic experience (Park et al., 2005, Annals of Surgery) recommended selective resection of Meckel's diverticulum based on four features: age less than 50, male sex, length greater than 2 cm, or presence of histologically abnormal tissue. — Nick Bruns summarizing the discussion [Ep 3 · 3:10](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=190)
- A Miami Children's Hospital paper (Al-Khoury et al., JAMA Surgery 2012) studied 200 consecutive non-perforated appendicitis patients. 80% were same-day discharges with similar complication and readmission rates compared to overnight stays. — Nick Bruns summarizing the discussion [Ep 3 · 4:28](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=268)
- Dr. Levitt has performed over 5,000 colorectal procedures. — Marc Levitt summarizing a resource [Ep 2 · 2:31](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=151)
- If going to OR for elective pull-through and sigmoid/left colon biopsies show no ganglion cells, should not proceed with pull-through that day; instead biopsy transverse colon and hepatic flexure, do ileostomy with frozen section confirmation, and return another day for definitive repair. — Philippa Jalius summarizing the discussion [Ep 6 · 19:30](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1170)
- Reinforcement layer is critical to line up bowel pieces for mucosa-to-mucosa anastomosis — Rod Gootee summarizing the discussion [Ep 4 · 17:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=1020)
- Contrast enema for Hirschsprung is only about 90% accurate in determining the level of aganglionosis. — Felipe Childish summarizing the discussion [Ep 10 · 5:38](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=338)
- According to Dane Teitelbaum's paper on reoperative Hirschsprung surgery, if you are not happy with permanent pathology, going back early is not a bad idea because adhesions are not yet firm and it is easier to redissect around the pull-through. — Martin Latcher summarizing a resource [Ep 10 · 9:21](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=561)
- The septic source in Hirschsprung disease is not the dilated bowel itself but the Hirschsprung enterocolitis, requiring IV Flagyl treatment. — Felipe Glu summarizing the discussion [Ep 5 · 9:20](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=560)

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