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

Updated: n/a · 14 episodes · 268 cited statements

## Episodes
### Foundations
- [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)
- [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)

### Surgical Management
- [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](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)
- [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)
- [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 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)

## Chapters
- [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 1)
- [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 1)
- [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 1)
- [5:05](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=305) Doctor Boley's Contributions and Personal Connections (Ep 1)
- [8:34](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=514) Doctor Henrys and Primary Pull-Through (Ep 1)
- [9:36](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=576) Doctor Martin's Contributions to Colorectal Surgery (Ep 1)
- [12:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=760) Modern Era: Noblet, Jorgeson, Langer, de la Torre, and Teitelbaum (Ep 1)
- [14:17](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13818?t=857) Closing Reflections (Ep 1)
- [0:00](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=0) Case Presentation: Septic Neonate with Trisomy 21 (Ep 7)
- [4:40](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=280) Initial Management: Irrigations and Antibiotics (Ep 7)
- [9:03](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=543) Diagnostic Confirmation: Contrast Enema and Rectal Biopsy (Ep 7)
- [12:20](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=740) Surgical Timing and Leveling Biopsy Strategy (Ep 7)
- [19:57](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1197) Ileostomy versus Colostomy Decision-Making (Ep 7)
- [24:08](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=1448) Enterocolitis Prevention: Botox and Patient Education (Ep 7)
- [0:08](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=8) Introduction and Guest Presentation (Ep 11)
- [2:16](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=136) Case Presentation: Late-Onset Hirschsprung with Staged Approach (Ep 11)
- [3:52](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=232) Biopsy Strategy and Surgical Technique (Ep 11)
- [5:54](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=354) Laparoscopic Port Placement and Mobilization (Ep 11)
- [7:43](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=463) Pathology Complication: Transition Zone Pull-Through (Ep 11)
- [8:52](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=532) Early Re-Operation Strategy and Outcome (Ep 11)
- [15:18](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=918) Biopsy Technique and Pathology Interpretation (Ep 11)
- [16:37](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-44-hd-frozen-section-13823?t=997) Surgical Wisdom: Stopping the Cascade (Ep 11)
- [0:00](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=0) Case Presentation: Two-Day-Old with Trisomy 21 and Abdominal Distension (Ep 6)
- [4:10](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=250) Initial Management Strategy and Perforation Patterns (Ep 6)
- [7:17](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=437) Irrigation Technique and Enterocolitis Pathophysiology (Ep 6)
- [9:39](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=579) Diagnostic Confirmation: Contrast Enema and Rectal Biopsy (Ep 6)
- [12:41](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=761) Timing of Definitive Surgery and Leveling Biopsy Strategy (Ep 6)
- [18:44](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1124) Ostomy Decision-Making: Ileostomy versus Colostomy (Ep 6)
- [24:15](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-13848?t=1455) Enterocolitis Prevention: Botox and Patient Education (Ep 6)
- [0:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=0) Introduction and Episode Setup (Ep 2)
- [0:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=26) Early Pioneers: Hirschsprung, Swenson, Yancey, Suave, Duhamel, Rabine, and Boley (Ep 2)
- [5:05](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=305) Personal Connections: Boley and Henry So (Ep 2)
- [9:36](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=576) Dr. Martin's Contributions: Long-Segment Technique and Ulcerative Colitis (Ep 2)
- [11:41](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=701) Modern Era: Noblet, Jorgensen, Langer, de la Torre, and Teitelbaum (Ep 2)
- [14:47](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-21-the-history-of-hirschsprung-disease-13861?t=887) Closing Remarks (Ep 2)
- [0:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=0) Introduction and case presentation (Ep 5)
- [2:26](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=146) Preoperative preparation and laparoscopic approach (Ep 5)
- [5:40](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=340) Port placement and biopsy technique (Ep 5)
- [9:00](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=540) Mesenteric dissection technique (Ep 5)
- [11:42](https://qa.library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-13864?t=702) Transanal dissection and pull-through (Ep 5)

## 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 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 3 · 21:27](https://qa.library.globalcastmd.com/watch/hirschsprung-13870?t=1287)
- The vast majority of Hirschsprung patients do extremely well after pull-through with normal emptying and bowel control. — Marc Levitt (clinical) [Ep 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 14 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 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 4 · 32:50](https://qa.library.globalcastmd.com/watch/hirschsprung-disease-pediacast-287-13877?t=1970)
- 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 2 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 5 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 1 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 11 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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 7 · 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.
- Dr. Levitt has performed over 5,000 colorectal procedures. — Marc Levitt summarizing a resource [Ep 4 · 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 5 · 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 11 · 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 11 · 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 7 · 9:20](https://qa.library.globalcastmd.com/watch/colorectal-quiz-episode-46-hirschsprung-s-disease-13821?t=560)

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