# Hirschsprung Disease — GCMD Library living collection Updated: n/a · 19 episodes · 429 cited statements ## Episodes ### Tools ### High-Yield Summaries - [Hirschsprung's Disease](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187) — podcast · 20:48 · [machine version](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187.md) - [Hirschsprung Disease in Brief](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023) — video · 10:44 · [machine version](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023.md) - [Colorectal Quiz Episode 3: Hirschsprung Disease](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649) — podcast · 20:10 · [machine version](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649.md) ### In-depth Review - [Hirschsprung Disease Part I with Marc Levitt](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311) — podcast · 59:20 · [machine version](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311.md) - [Hirschsprung Disease Part II with Dr. Marc Levitt](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310) — podcast · 44:38 · [machine version](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310.md) - [The Colorectal Quiz Episode 3.5: Proximal Hirschsprung Disease](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682) — podcast · 8:42 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682.md) - [The Colorectal Quiz Episode 4: Classic Hirschsprung disease - Surgical Technique](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704) — podcast · 19:28 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704.md) - [The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745) — podcast · 14:37 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745.md) - [The Colorectal Quiz Episode 10: Total Colonic Hirschsprung Disease Part 1](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893) — podcast · 12:24 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893.md) - [Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407) — podcast · 24:11 · [machine version](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407.md) - [The Colorectal Quiz Episode 20: Hirschsprung Disease Obstruction Part 2](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461) — podcast · 21:29 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461.md) - [The Colorectal Quiz Episode 22: Hirschsprung Disease - the Soiling Patient Part 1](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616) — podcast · 19:45 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616.md) - [The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient Part 2 -The Dentate Line and Motility](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739) — podcast · 26:46 · [machine version](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739.md) - [Colorectal Quiz Episode 27: Delayed Hirschsprung Disease](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052) — podcast · 21:40 · [machine version](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052.md) ### Work-up and Treatment - [Hirschsprung Disease Workup](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411) — podcast · 10:44 · [machine version](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411.md) ### Technique Videos/OP Notes - [Technique: Laparoscopic Assisted Pull-through for Hirschsprung's Disease](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756) — video · 10:47 · [machine version](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756.md) ### Updates - [Different Rectal Biopsy Techniques for Hirschsprung Disease](https://library.globalcastmd.com/watch/different-rectal-biopsy-techniques-for-hirschsprung-disease-4038) — article · [machine version](https://library.globalcastmd.com/watch/different-rectal-biopsy-techniques-for-hirschsprung-disease-4038.md) - [Inpatient management of Hirschsprung’s associated enterocolitis treatment: the benefits of standardized care](https://library.globalcastmd.com/watch/inpatient-management-of-hirschsprung-s-associated-enterocolitis-treatment-the-benefits-of-standardized-care-3085) — article · [machine version](https://library.globalcastmd.com/watch/inpatient-management-of-hirschsprung-s-associated-enterocolitis-treatment-the-benefits-of-standardized-care-3085.md) - [The Extent of the Transition Zone in Hirschsprung's Disease](https://library.globalcastmd.com/watch/the-extent-of-the-transition-zone-in-hirschsprung-s-disease-1386) — article · [machine version](https://library.globalcastmd.com/watch/the-extent-of-the-transition-zone-in-hirschsprung-s-disease-1386.md) ## Chapters - [0:00](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=0) Introduction and recap of previous trauma episode (Ep 5) - [2:20](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=140) Post-pull-through enterocolitis: presentation and pathophysiology (Ep 5) - [5:20](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=320) Anatomic causes of post-pull-through obstruction (Ep 5) - [8:20](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=500) Acute enterocolitis management protocol (Ep 5) - [10:22](https://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 5) - [12:41](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=761) Examination under anesthesia technique and biopsy (Ep 5) - [18:03](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1083) Surgical management of transition-zone pull-through (Ep 5) - [20:13](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1213) Surgical management of obstructing Soave cuff (Ep 5) - [23:43](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1423) Role of Botox in sphincter achalasia (Ep 5) - [27:10](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1630) Management of twisted pull-through (Ep 5) - [29:41](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1781) Redo of failed Duhamel pouch (Ep 5) - [32:35](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=1955) Evaluation of the soiling patient (Ep 5) - [36:15](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2175) Four clinical scenarios in soiling patients (Ep 5) - [42:39](https://library.globalcastmd.com/watch/hirschsprung-disease-part-ii-with-dr-marc-levitt-310?t=2559) Surgical options for incontinence and closing remarks (Ep 5) - [0:00](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=0) Introduction and Appendicitis Review (Ep 4) - [5:10](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=310) Initial Evaluation and Diagnosis of Hirschsprung Disease (Ep 4) - [12:36](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=756) Pathologic Confirmation and Surgical Timing (Ep 4) - [17:50](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1070) Historical Evolution of Surgical Techniques (Ep 4) - [27:53](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1673) Current Surgical Approach: Transanal Swenson (Ep 4) - [32:15](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=1935) Technical Details: Exposure and Dissection Plane (Ep 4) - [37:13](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2233) Laparoscopic Approach and Biopsy Technique (Ep 4) - [47:56](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=2876) Postoperative Management (Ep 4) - [56:08](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3368) Special Circumstance: Hepatic Flexure Transition Zone (Ep 4) - [0:00](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=0) Patient positioning, port placement, and biopsy (Ep 16) - [1:15](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=75) Laparoscopic mesenteric dissection to pelvic floor (Ep 16) - [4:35](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=275) Timing of surgery and completion of laparoscopic phase (Ep 16) - [5:10](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=310) Transanal mucosal dissection (Ep 16) - [7:31](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=451) Muscular cuff division and pull-through (Ep 16) - [8:57](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=537) Coloanal anastomosis and postoperative course (Ep 16) - [0:00](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=0) Introduction and epidemiology of Hirschsprung disease (Ep 1) - [1:55](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=115) Diagnostic workup: contrast enema, rectal biopsy, and anorectal manometry (Ep 1) - [5:08](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=308) Initial management after diagnosis (Ep 1) - [6:55](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=415) Surgical principles and techniques: Swenson, Suave, and Duhamel procedures (Ep 1) - [11:46](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=706) Complications and Hirschsprung-associated enterocolitis (Ep 1) - [13:51](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=831) Long-term prognosis and management of poor outcomes (Ep 1) - [17:05](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=1025) Clinical pearls for trainees (Ep 1) - [0:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=0) Introduction and Series Context (Ep 3) - [1:17](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=77) Case Presentation and Differential Diagnosis (Ep 3) - [3:47](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=227) Imaging Workup and Contrast Enema Indications (Ep 3) - [7:16](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=436) Contrast Enema Interpretation (Ep 3) ## Statements Every statement is attributed, typed, and timestamped; the link is the citation. - The vast majority of Hirschsprung patients do extremely well post-pull-through with normal emptying and bowel control. — Marc Levitt (clinical) [Ep 5 · 3:29](https://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 5 · 3:37](https://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 5 · 4:25](https://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 5 · 4:59](https://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 5 · 5:20](https://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 5 · 5:38](https://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 5 · 5:50](https://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 5 · 7:14](https://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 5 · 8:20](https://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 5 · 10:04](https://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 5 · 11:21](https://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 5 · 11:32](https://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 5 · 12:13](https://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 5 · 13:09](https://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 5 · 16:34](https://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 5 · 17:02](https://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 5 · 17:30](https://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 5 · 18:17](https://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 5 · 18:27](https://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 5 · 19:25](https://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 5 · 20:31](https://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 5 · 21:24](https://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 5 · 21:51](https://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 5 · 22:09](https://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 5 · 24:34](https://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 5 · 24:51](https://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 5 · 25:19](https://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 5 · 25:35](https://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 5 · 26:07](https://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 5 · 26:45](https://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 5 · 27:17](https://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 5 · 28:01](https://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 5 · 30:18](https://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 5 · 31:00](https://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 5 · 33:15](https://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 5 · 33:48](https://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 5 · 34:03](https://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 5 · 34:28](https://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 5 · 35:04](https://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 5 · 35:36](https://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 5 · 36:19](https://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 5 · 37:06](https://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 5 · 37:43](https://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 5 · 39:12](https://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 5 · 40:35](https://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 5 · 41:13](https://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 5 · 42:30](https://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 5 · 43:00](https://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 4 · 8:43](https://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 4 · 8:55](https://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 4 · 12:38](https://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 4 · 13:23](https://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 4 · 14:59](https://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 4 · 15:42](https://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 4 · 18:14](https://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 4 · 19:28](https://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 4 · 20:18](https://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 4 · 21:18](https://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 4 · 22:19](https://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 4 · 23:21](https://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 4 · 25:34](https://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 4 · 26:23](https://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 4 · 27:06](https://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 4 · 28:50](https://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 4 · 29:18](https://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 4 · 31:18](https://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 4 · 34:09](https://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 4 · 35:30](https://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 4 · 36:39](https://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 4 · 38:19](https://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 4 · 38:45](https://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 4 · 40:56](https://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 4 · 44:03](https://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 4 · 44:20](https://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 4 · 45:57](https://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 4 · 47:02](https://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 4 · 53:23](https://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 4 · 54:39](https://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 4 · 55:52](https://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 4 · 56:31](https://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 4 · 57:05](https://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 4 · 58:02](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=3482) - The key to mesenteric dissection is staying exactly adjacent to the colon wall to prevent injury to surrounding structures. (clinical) [Ep 16 · 1:21](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=81) - Because all heat and energy remains between the jaws of the sealer, there is no danger in injuring surrounding structures by brushing aside them with the sealer. (clinical) [Ep 16 · 1:32](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=92) - Small perforating vessels are grasped, sealed, and pulled down off the rectal wall circumferentially to dissect along the aganglionic portion of the colon. (clinical) [Ep 16 · 1:48](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=108) - The advantage of the 3-millimeter sealer is that it can both dissect off vessels and dissect around the colon to mobilize tissue, with no risk of pass-pointing as with a 3-millimeter hook. (clinical) [Ep 16 · 2:32](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=152) - There is no need to perform instrument changes with the right hand throughout the case; a bowel grasper is in the left hand and the sealer is the only instrument used in the right hand. (clinical) [Ep 16 · 2:55](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=175) - Carrying the dissection down to the pelvic floor limits the amount of transanal dissection necessary and decreases the risk of injury to the external sphincter. (clinical) [Ep 16 · 3:24](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=204) - Because there is no energy spread from the tips of the instrument, it is safe for the instrument to be adjacent to the bladder, vagina, prostate, and other surrounding structures. (clinical) [Ep 16 · 3:51](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=231) - Energy only between the jaws of the instrument diminishes the risk of injury to the ureters and other vital structures such as the vas deferens. (clinical) [Ep 16 · 4:05](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=245) - When diagnosed, the preference is to perform this operation in the newborn period prior to discharge to home, as the operation is extremely safe with current technology. (opinion) [Ep 16 · 4:35](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=275) - It is acceptable if the child tolerates rectal irrigations to let them grow before surgery. (clinical) [Ep 16 · 4:40](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=280) - A series of traction stitches are placed inside the anus just proximal to the dentate line and then out to the skin, slightly inverting the anus so the dentate line can be clearly visualized; 4 to 8 sutures are used. (clinical) [Ep 16 · 5:13](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=313) - A mucosal incision is made with handheld cautery 2 to 3 millimeters proximal to the dentate line. (clinical) [Ep 16 · 5:53](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=353) - Traction or stay sutures are placed in the mucosa to help retract it and allow for submucosal dissection. (clinical) [Ep 16 · 6:12](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=372) - The key to the transanal portion is that it should all take place externally to the anus, with no retractors ever placed within the external sphincter, which may cause these muscles to be damaged. (clinical) [Ep 16 · 6:28](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=388) - The beauty of the laparoscopic dissection down to the pelvic floor is that it allows release of the area so dissection can be carried out outside of the anus, protecting the external sphincter muscles and improving the chance of good continence. (clinical) [Ep 16 · 6:36](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=396) - Sharp dissection is preferred to mobilize the submucosal space, although blunt dissection can also be used. (opinion) [Ep 16 · 7:05](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=425) - The dissection is carried around circumferentially until the muscular cuff everts, at which point the peritoneal cavity is entered. (clinical) [Ep 16 · 7:20](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=440) - A stitch is placed as a retractor so the muscular cuff can be divided at the 6 o'clock position, and the dissection is carried circumferentially, allowing the colon to be pulled down through the anus. (clinical) [Ep 16 · 8:00](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=480) - The biopsy site is 5 to 6 centimeters above the obvious transition zone. (clinical) [Ep 16 · 8:19](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=499) - Remaining mesenteric attachments are taken externally using the 3-millimeter sealer to allow mobilization 5 to 6 centimeters above the biopsy site. (clinical) [Ep 16 · 8:27](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=507) - Laparoscopy is used to ensure proper orientation of the pull-through and that no structures are caught under the mesentery. (clinical) [Ep 16 · 8:48](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=528) - The colon is divided 5 to 6 centimeters above the biopsy site, which is 5 centimeters above the obvious transition zone. (clinical) [Ep 16 · 8:57](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=537) - Four quadrant stay sutures are placed, and 3 to 4 additional sutures are placed in each quadrant for a total of 12 to 16 sutures forming the new coloanal anastomosis, using 4-0 Vicryl suture in newborns. (clinical) [Ep 16 · 9:19](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=559) - This operation took 70 minutes. (clinical) [Ep 16 · 9:57](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=597) - The child was left without a nasogastric tube and started stooling the morning following surgery. (clinical) [Ep 16 · 10:00](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=600) - The patient was started on feeds less than 24 hours after the procedure. (clinical) [Ep 16 · 10:07](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=607) - The anastomosis is calibrated with a number 12 Hegar dilator at the end of the procedure. (clinical) [Ep 16 · 10:36](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=636) - A gauze packing is placed in the anus at the end of the procedure. (clinical) [Ep 16 · 10:45](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=645) - Hirschsprung disease is a congenital developmental anomaly of intestinal ganglion cell migration that results in a functional bowel obstruction. — Aaron Garrison (clinical) [Ep 1 · 0:07](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=7) - More than 95% of neonates pass meconium within the first 48 hours of life, and failure to pass meconium is typical of Hirschsprung's disease. — Jason Frischer (clinical) [Ep 1 · 0:35](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=35) - Some infants with Hirschsprung's disease do pass meconium. — Jason Frischer (clinical) [Ep 1 · 0:35](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=35) - Hirschsprung disease has an incidence of about 1 in 5,000 children. — Aaron Garrison (epidemiological) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - About 10% of children with Hirschsprung disease will have a positive family history. — Aaron Garrison (epidemiological) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - There are predisposing genetic conditions such as the RET gene that can be associated with Hirschsprung disease. — Aaron Garrison (clinical) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - Up to 10% of children with Hirschsprung's disease will have trisomy 21. — Aaron Garrison (epidemiological) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - Only 1 to 2% of patients with trisomy 21 have Hirschsprung disease. — Aaron Garrison (epidemiological) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - Hirschsprung disease is associated with Waardenburg syndrome, congenital central hypoventilation (Ondine's curse), and some other syndromes. — Aaron Garrison (clinical) [Ep 1 · 1:03](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=63) - The classic finding on contrast enema is a transition zone in the rectosigmoid, but that transition zone can be located anywhere within the bowel. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - A rectosigmoid ratio of less than 1.0 is suggestive of Hirschsprung's disease, meaning the aganglionic bowel is contracted and narrow compared to the dilated ganglionated bowel more proximal. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - In total colonic Hirschsprung's disease, a foreshortened or question mark colon can be seen on contrast enema. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - Approximately 10% of newborns with Hirschsprung's disease may lack the typical findings on a contrast enema. — Jason Frischer (epidemiological) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - Rectal biopsy is the true definitive diagnosis for Hirschsprung disease. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - Typical features on rectal biopsy include absence of ganglion cells, presence of hypertrophic nerves, abnormal pattern of cholinesterase staining, and an absence of calretinine staining. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - To be considered an adequate biopsy, it must be taken from the rectum at least one centimeter above the dentate line and must include both mucosa and submucosal layers. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - Suction biopsy technique is typically used for patients less than six months of age. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - Full thickness biopsy technique should be considered for patients older than six months, or when a suction biopsy is inadequate. — Jason Frischer (clinical) [Ep 1 · 1:57](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=117) - In Hirschsprung's disease, anorectal manometry shows a lack of the rectoanal inhibitory reflex (RAIR). — Jason Frischer (clinical) [Ep 1 · 4:23](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=263) - The rectoanal inhibitory reflex may be absent in other conditions as well, and some children have a false positive test. — Jason Frischer (clinical) [Ep 1 · 4:23](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=263) - Any patient with an absent RAIR must undergo a rectal biopsy for confirmation of the diagnosis. — Jason Frischer (guideline) [Ep 1 · 4:23](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=263) - Enterocolitis is the life-threatening part of Hirschsprung's disease. — Aaron Garrison (clinical) [Ep 1 · 5:12](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=312) - NICU babies diagnosed with Hirschsprung's disease are typically managed with irrigations, antibiotics if they show evidence of enterocolitis, and NPO or NG tubes if they're distended. — Aaron Garrison (clinical) [Ep 1 · 5:12](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=312) - Older children with Hirschsprung disease often have dilated colon and are not amenable to just doing a primary pull-through in many cases. — Aaron Garrison (clinical) [Ep 1 · 5:12](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=312) - The three goals of surgical management are: identify the extent of the aganglionic segment, resect that segment, and restore the bowel to its continuity. — Jason Frischer (clinical) [Ep 1 · 6:42](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=402) - The contrast enema can be used as a roadmap for surgery, and most times it is accurate to where the level is, but not always. — Aaron Garrison (clinical) [Ep 1 · 6:59](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=419) - The goal of surgery is to get past the aganglionic segment into the normally innervated bowel that does not have hypertrophic nerves, and to pull that segment of bowel down through and perform anastomosis above the dentate line. — Aaron Garrison (clinical) [Ep 1 · 6:59](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=419) - In the Swenson procedure, you pull down the aganglionic bowel and perform a full thickness anastomosis one to two centimeters above the dentate line, leaving the most minimal amount of aganglionic bowel possible. — Jason Frischer (clinical) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - In the Swenson procedure, it is very important to be cautious about injuring the urethra as it's quite close, especially in boys. — Jason Frischer (clinical) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - The Suave procedure involves performing a mucosectomy from a transanal approach slightly above the dentate line and pulling through the normal bowel. — Jason Frischer (clinical) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - In the Suave procedure, the actual anastomosis is performed within a cuff of aganglionic rectum. — Jason Frischer (clinical) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - The Suave procedure theoretically causes less injury to pelvic structures, nerves, urethra and vagina. — Jason Frischer (opinion) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - A con of the Suave procedure is that the aganglionic cuff can become stiff enough to cause an obstruction or outlet obstruction. — Jason Frischer (clinical) [Ep 1 · 7:36](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=456) - In the Duhamel procedure, you intentionally leave a portion of the aganglionic rectum behind and bring the normally ganglionated bowel posterior to that rectum, then make an anastomosis using a stapler to create a common panel. — Aaron Garrison (clinical) [Ep 1 · 10:15](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=615) - The Duhamel procedure involves less dissection in the pelvis, so there may be less injury to pelvic structures. — Aaron Garrison (opinion) [Ep 1 · 10:15](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=615) - Children who have the Duhamel procedure may have higher risks of constipation and stool withholding because they can have trouble evacuating the pouch. — Aaron Garrison (clinical) [Ep 1 · 10:15](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=615) - In the Duhamel procedure, the anastomosis can leave a spur if it is not large enough, sometimes requiring revision. — Aaron Garrison (clinical) [Ep 1 · 10:15](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=615) - All three surgical techniques (Swenson, Suave, and Duhamel) are equally effective. — Aaron Garrison (opinion) [Ep 1 · 10:15](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=615) - Early complications after Hirschsprung surgery include diaper rash and excoriation that can need to be treated like a burn. — Aaron Garrison (clinical) [Ep 1 · 11:49](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=709) - Anastomotic leaks are rare but usually show up in the first week after surgery with fever, abdominal distension, and possibly free air, often requiring another operation or proximal diversion. — Aaron Garrison (clinical) [Ep 1 · 11:49](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=709) - Hirschsprung's-associated enterocolitis is likely an inflammatory condition secondary to bacterial overgrowth. — Jason Frischer (clinical) [Ep 1 · 12:34](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=754) - A child with enterocolitis presents with abdominal distension, vomiting, fever, and change in bowel habits that could be either diarrhea or not passing stool at all. — Jason Frischer (clinical) [Ep 1 · 12:34](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=754) - Treatment of enterocolitis includes fluid resuscitation, digital rectal exam, and colonic irrigations. — Jason Frischer (clinical) [Ep 1 · 12:34](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=754) - For enterocolitis, antibiotics including metronidazole or broad-spectrum antibiotics are added depending on the severity of presentation. — Jason Frischer (clinical) [Ep 1 · 12:34](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=754) - About 80% of kids with Hirschsprung's disease are constipated and will need some kind of management. — Aaron Garrison (epidemiological) [Ep 1 · 13:54](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=834) - Most patients with rectosigmoid Hirschsprung disease should be continent by the time they enter kindergarten. — Aaron Garrison (clinical) [Ep 1 · 13:54](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=834) - For patients not doing well after pull-through, if there is a stricture at the anastomosis, it needs to be addressed either through dilations or revising the pull through procedure. — Jason Frischer (clinical) [Ep 1 · 14:51](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=891) - Anatomic problems after pull-through can include a twist in the pull through, an obstructing cuff, a non-functioning Duhamel pouch, or a transition zone or continued aganglionic segment. — Jason Frischer (clinical) [Ep 1 · 14:51](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=891) - For patients with anatomic problems after pull-through, you typically have to revise the pull through. — Jason Frischer (clinical) [Ep 1 · 14:51](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=891) - For patients without anatomic problems after pull-through, treatment can include bowel management, Botox injection to relieve sphincter tone, or motility studies. — Jason Frischer (clinical) [Ep 1 · 14:51](https://library.globalcastmd.com/watch/hirschsprung-s-disease-3187?t=891) - The plain abdominal x-ray showed a big right colon, prominent transverse colon, and a compressed left colon with small lumen, along with possible small bowel dilation. (clinical) [Ep 6 · 3:16](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=196) - It is hard on a newborn film to really discern small and large bowel, and you can get fooled. — Jason Frischer (clinical) [Ep 6 · 3:48](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=228) - A baby with bilious emesis and non-classic abdominal x-ray could have had distal air and then had a volvulus, requiring urgent upper GI to rule out malrotation before contrast enema. — Todd Ponsky (clinical) [Ep 6 · 4:05](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=245) - A limited upper GI was performed and ruled out malrotation in this child. — Jason Frischer (clinical) [Ep 6 · 4:43](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=283) - The contrast enema showed an impressive right colon with transverse colon tapering off, and a transition zone probably somewhere in the transverse colon. (clinical) [Ep 6 · 5:01](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=301) - Patients with proximal Hirschsprung disease are at risk of perforation, usually in the cecum. — Jason Frischer (clinical) [Ep 6 · 5:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=320) - To get a perforation, you need distension, and if you have a transition zone at the hepatic flexure, then all the pressure is in the right colon. — Jason Frischer (clinical) [Ep 6 · 5:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=320) - A baby that doesn't have a competent ileosecal valve might be saved from perforation because pressure can decompress into the small bowel. — Jason Frischer (clinical) [Ep 6 · 5:41](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=341) - You are obligated at some point, maybe after resuscitation, to get a rectal biopsy in a patient with suspected Hirschsprung disease. — Jason Frischer (clinical) [Ep 6 · 5:57](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=357) - If the transition zone is at the hepatic flexure, you can predict that enough pressure builds up in the right colon to have the cecum perforate. (clinical) [Ep 6 · 6:04](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=364) - You rarely get a perforation in a more standard sigmoid level transition zone. (clinical) [Ep 6 · 6:04](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=364) - A suction rectal biopsy confirmed the diagnosis of Hirschsprung disease in this patient. (clinical) [Ep 6 · 6:58](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=418) - Proximal Hirschsprung disease and distal Hirschsprung disease require two different operative approaches. — Jason Frischer (clinical) [Ep 6 · 7:14](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=434) - Maternal magnesium sulfate used to slow delivery can cause neonatal abdominal distention that mimics Hirschsprung disease — Levitt (clinical) [Ep 3 · 2:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=163) - Maternal opiates can cause neonatal abdominal distention mimicking Hirschsprung disease — Levitt (clinical) [Ep 3 · 2:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=163) - Hypothyroidism can present with neonatal abdominal distention similar to Hirschsprung disease — Levitt (clinical) [Ep 3 · 2:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=163) - Small left colon syndrome associated with maternal diabetes can mimic Hirschsprung disease — Levitt (clinical) [Ep 3 · 2:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=163) - Contrast enema should not be performed in the presence of enterocolitis due to risk of perforation — Levitt (clinical) [Ep 3 · 6:44](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=404) - Rectal irrigations before contrast enema do not change the result of the contrast study — Levitt (opinion) [Ep 3 · 7:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=453) - The contrast study serves as a roadmap for surgery rather than a definitive diagnostic tool for Hirschsprung disease — Levitt (clinical) [Ep 3 · 7:33](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=453) - In Hirschsprung disease, the aganglionic rectum appears narrow due to spasm and inability to relax, while the ganglionated proximal bowel is dilated, creating the recto-sigmoid ratio — Jason Frischer (clinical) [Ep 3 · 9:52](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=592) - Hyperperistalsis and tortuosity in the rectum on contrast enema is a classic finding in Hirschsprung disease, reflecting hypercontractility of the aganglionic segment — Levitt (clinical) [Ep 3 · 10:30](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=630) - The exact location of the transition zone cannot be precisely determined on contrast study — Levitt (clinical) [Ep 3 · 10:30](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=630) - Suction rectal biopsy is appropriate for neonates and does not require open biopsy in the operating room — Levitt (clinical) [Ep 3 · 12:46](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=766) - Three good biopsy specimens should be obtained for pathologic evaluation — Jason Frischer (clinical) [Ep 3 · 13:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=783) - Quick diff staining highlights ganglion cells better than standard H&E staining — Jason Frischer (clinical) [Ep 3 · 13:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=783) - The diagnostic criteria for Hirschsprung disease at the Washington institution is absence of ganglion cells in 100 pathologic levels — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - If a single ganglion cell is found on biopsy, the diagnosis is not Hirschsprung disease regardless of ganglion cell density — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - Nerve trunk hypertrophy is defined as nerve trunks greater than 40 microns in diameter — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - Pathologists should measure nerve trunks in rectal biopsies to confirm hypertrophy — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - A pathology report showing no ganglion cells without comment on nerve hypertrophy is not satisfactory for surgical decision-making — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - Patients have been incorrectly operated on for Hirschsprung disease based on pathology reports showing no ganglion cells but lacking nerve hypertrophy documentation — Levitt (clinical) [Ep 3 · 14:27](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=867) - Everyone is physiologically aganglionic in the zone of the anal canal — Levitt (clinical) [Ep 3 · 15:40](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=940) - Hypertrophic nerves are not found in the anal canal zone, so absence of ganglion cells there without nerve hypertrophy does not indicate Hirschsprung disease — Levitt (clinical) [Ep 3 · 15:40](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=940) - Presence of squamous epithelium in a rectal biopsy confirms the biopsy was taken too low (in the anal canal) — Levitt (clinical) [Ep 3 · 15:40](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=940) - The optimal location for rectal biopsy is 0.5 to 1 cm above the crypts to ensure columnar epithelium — Levitt (clinical) [Ep 3 · 15:40](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=940) - The crypts are located above the dentate line, so optimal biopsy location is at least 1-2 cm above the dentate line — Jason Frischer (clinical) [Ep 3 · 16:45](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=1005) - Infants presenting with Hirschsprung-like symptoms who have ganglion cells but numerous eosinophils on biopsy may have allergic colitis — Levitt (clinical) [Ep 3 · 16:55](https://library.globalcastmd.com/watch/colorectal-quiz-episode-3-hirschsprung-disease-3649?t=1015) - The most important reason to use laparoscopy for Hirschsprung pull-through is to achieve deep pelvic dissection, minimizing transanal work and avoiding overstretching of the sphincters, which is a significant source of morbidity. — Marc Levitt (clinical) [Ep 7 · 6:01](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=361) - With proper laparoscopic dissection, the transanal portion of a primary Hirschsprung pull-through should take well under one hour. — Jason Frischer (clinical) [Ep 7 · 6:37](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=397) - For surgeons without laparoscopy available, an umbilical approach can accomplish significant dissection work. — Marc Levitt (clinical) [Ep 7 · 6:48](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=408) - Full-thickness biopsy should be cut as a cube with the seromuscular side equal to the mucosal side to ensure adequate tissue for pathology evaluation. — Marc Levitt (clinical) [Ep 7 · 7:37](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=457) - Surgeons should wait for frozen section confirmation before taking mesentery during Hirschsprung pull-through. — Aaron Garrison (clinical) [Ep 7 · 9:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=570) - Pathology must confirm presence of ganglion cells and nerves less than 40 microns in diameter before proceeding with pull-through. — Andrea Badillo (clinical) [Ep 7 · 10:08](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=608) - The biopsy must include submucosa because ganglion cells may be present in the seromuscular layer while hypertrophic nerves are present in the submucosal layer. — Marc Levitt (clinical) [Ep 7 · 10:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=620) - Mesenteric dissection should stay close to the bowel wall, not deep in the mesentery, as this plane tends to be less bloody. — Aaron Garrison (clinical) [Ep 7 · 11:13](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=673) - Staying close to the bowel during distal rectal dissection is critical because the old Swenson technique with wide dissection resulted in incontinence and urinary retention, likely from injury to the nerve erigentis. — Marc Levitt (clinical) [Ep 7 · 11:52](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=712) - For distal Hirschsprung disease, only distal branches of the IMA need to be taken, but for left colon involvement, the IMA itself may need to be taken to achieve adequate reach. — Jason Frischer (clinical) [Ep 7 · 12:26](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=746) - The transanal dissection should begin 1 cm above the dentate line to protect the dentate line and sphincters from injury. — Andrea Badillo (clinical) [Ep 7 · 13:04](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=784) - Lone Star retractor pins should be placed in three positions: first at the skin to identify the dentate line, then advanced to cover the dentate line, then moved to the mucosal opening site as dissection proceeds superiorly. — Andrea Badillo (clinical) [Ep 7 · 13:24](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=804) - The Swenson full-thickness dissection in the areolar plane is essentially bloodless and is preferred over submucosal dissection. — Marc Levitt (opinion) [Ep 7 · 14:12](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=852) - If a Soave submucosal dissection with cuff is performed, the cuff should be very short (approximately 1 cm) and must be split. — Marc Levitt (clinical) [Ep 7 · 14:32](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=872) - For standard rectosigmoid Hirschsprung cases, the patient can remain supine with legs wrapped and fastened to the ether screen, avoiding the need to flip prone. — Aaron Garrison (clinical) [Ep 7 · 15:03](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=903) - The resection margin should be approximately 5 cm above the biopsy site where the bowel appears healthy. — Andrea Badillo (clinical) [Ep 7 · 16:06](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=966) - Tacking sutures on the serosa to the pelvic sidewall at 3 and 9 o'clock positions help anchor the anastomosis in place, though this does not constitute a true two-layer anastomosis. — Aaron Garrison (clinical) [Ep 7 · 16:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=990) - Hypertrophic nerves are mainly a sacral nerve plexus entity, found in sigmoid and rectum, not in more proximal colon. (clinical) [Ep 8 · 5:48](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=348) - If frozen sections show no ganglion cells at the splenic flexure, do not proceed with pull-through that day; wait for permanent sections. (guideline) [Ep 8 · 9:32](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=572) - Ileostomy is preferred over colostomy when uncertain about transition zone level because ileostomy will almost definitely divert successfully. (opinion) [Ep 8 · 6:37](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=397) - When performing ileostomy for uncertain proximal disease, send a biopsy from the ileum to confirm it is ganglionic. (guideline) [Ep 8 · 6:49](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=409) - Mark biopsy sites with permanent suture using different numbers of tails for each site and document in operative report. (guideline) [Ep 8 · 7:29](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=449) - If you have even a little bit of colon, if you have your right colon, you can make one formed stool per day. (clinical) [Ep 8 · 10:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=620) - For mid-transverse colon transition zone, the pull-through blood supply is based on right colic vessels, and the middle colic must be ligated. — Andrea Badillo (clinical) [Ep 8 · 11:15](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=675) - To bring mid-transverse colon down for pull-through, the bowel must be de-rotated to avoid bringing the mesentery across the duodenum and creating obstruction. — Andrea Badillo (clinical) [Ep 8 · 11:21](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=681) - For transverse colon pull-through, ligate middle colic and very likely right colic; blood supply depends on ileocolic and the marginal artery paralleling the right colon. (clinical) [Ep 8 · 11:38](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=698) - De-rotation for transverse colon pull-through places the cecum at the liver bed, brings the pull-through down the right side, and puts small bowel on the left side — opposite rotation from Ladd's procedure. (clinical) [Ep 8 · 11:43](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=703) - When pulling transverse colon down, there is a slight twist in the mesentery, so ensuring adequate blood supply without kinking is critical. (clinical) [Ep 8 · 12:10](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=730) - Preference is to perform proximal Hirschsprung pull-through open, possibly through the ileostomy closure incision, though some do it laparoscopically. (opinion) [Ep 8 · 12:24](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=744) - There are functional outcome differences between pulling through transverse colon versus left-sided colon. (clinical) [Ep 8 · 9:45](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=585) - If planning to wait several months before pull-through and not diverting the colon, consider whether the colon needs to be beaten (decompressed), though the answer is uncertain. (opinion) [Ep 8 · 8:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=487) - Most defunctionalized colons can stay without needing irrigation access; only a small population will need the colon addressed if it becomes severely backed up with chalky stool. — Andrea Badillo (clinical) [Ep 8 · 8:46](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=526) - In settings without pathology support, empiric diversion in the dilated segment is a reasonable strategy; if that bowel works, that is where the pull-through will go. (guideline) [Ep 8 · 7:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=427) - If doing primary pull-through several days after mapping (waiting for permanent sections), ensure the child is doing well with irrigations and not having smoldering enterocolitis, which would warrant immediate diversion. (guideline) [Ep 8 · 6:09](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=369) - The nightmare scenario is not finding ganglion cells on frozen section (especially when not finding nerves) when in fact ganglion cells are present, leading to unnecessary resection of good colon. (clinical) [Ep 8 · 5:35](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=335) - 10% of newborns with meconium plug have Hirschsprung disease — Marc Levitt (epidemiological) [Ep 9 · 3:13](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=193) - Newborns with meconium plug should receive biopsy for Hirschsprung disease to avoid missing the diagnosis and having the child suffer months of constipation, poor feeding, and distension — Marc Levitt (clinical) [Ep 9 · 3:13](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=193) - In a newborn with meconium plug and Hirschsprung disease, the initial contrast enema shows what appears to be a meconium plug but is actually a segment of Hirschsprung disease — Marc Levitt (clinical) [Ep 9 · 3:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=210) - In total colonic Hirschsprung disease, contrast enema shows an amorphous, cylindrical colon without the classic narrowing at the rectum compared to the sigmoid seen in typical Hirschsprung disease — Marc Levitt (clinical) [Ep 9 · 6:33](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=393) - The age cutoff for switching from suction rectal biopsy to full-thickness biopsy is around 6 months — Jason Frischer (clinical) [Ep 9 · 7:26](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=446) - At 10 months of age, full-thickness biopsy is preferred over suction biopsy to ensure a definitive diagnosis — Marc Levitt (clinical) [Ep 9 · 7:39](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=459) - Suction biopsies are problematic when they do not provide a definitive diagnosis, requiring a subsequent trip to the OR for formal biopsy — Marc Levitt (clinical) [Ep 9 · 7:46](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=466) - If the patient is doing well and can be managed with irrigations, it is preferable to have final pathology diagnosis of Hirschsprung disease from rectal biopsy before entering the abdomen — Jason Frischer (clinical) [Ep 9 · 8:38](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=518) - Standard biopsy locations during operative mapping include rectosigmoid, proximal sigmoid around the left colon, splenic flexure/transverse colon, and right colon or hepatic flexure area — Jason Frischer (clinical) [Ep 9 · 9:13](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=553) - Post-pull-through obstruction can be caused by stricture, twist (any pull-through type), Soave cuff (Soave procedure), Duhamel spur or non-functional distended segment (Duhamel procedure), or non-functional segment (Rehbein procedure). — Hira Ahmad (clinical) [Ep 10 · 6:45](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=405) - In obstructed Hirschsprung patients, the colon fills with liquid stool with severe bacterial overgrowth, causing fluid loss into the bowel lumen, hypovolemia, and bacterial translocation/bacteremia, all occurring without passage of stool. — Marc Levitt (clinical) [Ep 10 · 10:10](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=610) - Enterocolitis can occur before surgery, after surgery, and even after successful surgery in babies who don't relax their sphincters and hold stool so efficiently they develop enterocolitis. — Marc Levitt (clinical) [Ep 10 · 11:06](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=666) - For a patient with prior Hirschsprung diagnosis presenting sick, assume Hirschsprung-associated enterocolitis; treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis is different (e.g., Crohn's disease, E. coli enterocolitis). — Jason Frischer (clinical) [Ep 10 · 12:06](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=726) - Post-pull-through enterocolitis within the first 3 months occurs in about 20% of patients, based on a study from Cincinnati and Columbus. — Marc Levitt (epidemiological) [Ep 10 · 13:58](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=838) - Families should not undergo Hirschsprung pull-through surgery until they can demonstrate ability to perform rectal irrigations, and they should be discharged with supplies to perform irrigations at home. — Jason Frischer (guideline) [Ep 10 · 14:58](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=898) - In rare circumstances where a patient is too ill and sedation in the ER doesn't work, go to the OR under general anesthesia for irrigation until the patient improves; occasionally an ileostomy is needed to get the child out of trouble and work up the pull-through problem later. — Marc Levitt (clinical) [Ep 10 · 16:30](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=990) - On contrast enema, it is important to look at the presacral space (space between the hollow of the sacrum and the pull-through); a widened presacral space is an abnormal finding. — Marc Levitt (clinical) [Ep 10 · 19:14](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=1154) - On contrast enema, if the catheter is inserted too high or the balloon is under too much pressure, distal pathology cannot be easily ascertained. — Rebecca Rentea (clinical) [Ep 10 · 20:48](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=1248) - A lateral view on contrast enema is important because it provides significant information about the presacral space and distal rectum anatomy. — Jason Frischer (clinical) [Ep 10 · 19:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=1183) - The anatomy of the original pull-through can explain the patient's obstructive symptoms, and it is important to know what pull-through type was performed to determine if there is a fixable problem. — Marc Levitt (clinical) [Ep 10 · 3:06](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=186) - If the original operative note is unavailable, a contrast study can help infer the original surgery type based on imaging findings; expertise in reading post-pull-through contrast studies is essential. — Marc Levitt (clinical) [Ep 10 · 7:42](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=462) - In a very ill child, a contrast study would not be the best initial option; resuscitation and stabilization should come first. — Rebecca Rentea (clinical) [Ep 10 · 8:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=505) - When performing initial rectal exam on a distended child with suspected enterocolitis, step to the side because there may be an explosive release of stool and gas. — Jason Frischer (clinical) [Ep 10 · 4:51](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=291) - In Hirschsprung patients with distention, irritability, and fever, enterocolitis should be assumed until proven otherwise. — Marc Levitt (clinical) [Ep 10 · 10:39](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=639) - Irrigation is the best way to break the cycle of enterocolitis because patients are not passing stool due to distal obstruction. — Marc Levitt (clinical) [Ep 10 · 10:55](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=655) - When reading literature on Hirschsprung enterocolitis rates, be aware that definitions vary (admission, need for irrigations, need for antibiotics); the PCPLC consortium is working on validating the Langer score for uniform application. — Marc Levitt (opinion) [Ep 10 · 13:31](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=811) - After pull-through surgery, wait one to two weeks before performing the first irrigation, and the first irrigation should be performed by someone who is confident and knows where the anastomosis is. — Marc Levitt (clinical) [Ep 10 · 14:31](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=871) - In a logical workup, obtain the contrast study before going to the OR for rectal exam under anesthesia, as it may reveal findings that guide the surgical approach. — Jason Frischer (clinical) [Ep 10 · 18:19](https://library.globalcastmd.com/watch/colorectal-quiz-episode-19-hirschsprung-disease-the-obstructed-patient-part-1-4407?t=1099) - Hirschsprung disease is a congenital condition affecting the lower most aspect of the intestine (rectum or sigmoid), characterized by missing ganglion cells in the submucosal and myenteric plexus that allow the intestine to relax. — Nelson Rosen (clinical) [Ep 15 · 1:05](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=65) - In Hirschsprung disease, the rectum and lowest part of the colon are always affected, with the aganglionosis always ending right above the anus. — Nelson Rosen (clinical) [Ep 15 · 2:00](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=120) - About 85% of Hirschsprung cases begin in the very end part of the sigmoid colon or the beginning of the rectum. — Nelson Rosen (epidemiological) [Ep 15 · 2:25](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=145) - In about 10% of Hirschsprung cases, the entire colon is affected. — Nelson Rosen (epidemiological) [Ep 15 · 2:35](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=155) - 90 to 95% of Hirschsprung cases are recognized in the newborn period. — Patty Curran (epidemiological) [Ep 15 · 2:52](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=172) - The first sign of Hirschsprung disease in newborns is usually failure to pass stool (meconium) within the first 24 to 48 hours. — Patty Curran (clinical) [Ep 15 · 3:00](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=180) - After failure to pass meconium, newborns with Hirschsprung disease develop bloating, continued failure to pass stool, and vomiting. — Patty Curran (clinical) [Ep 15 · 3:15](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=195) - In newborns with suspected Hirschsprung disease, the initial workup includes a water-soluble contrast enema looking for a narrower distal segment (rectum) with dilation above. — Nelson Rosen (clinical) [Ep 15 · 3:30](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=210) - If contrast enema raises concern for Hirschsprung disease, a suction rectal biopsy is performed to examine tissue for the presence of ganglion cells. — Nelson Rosen (clinical) [Ep 15 · 4:05](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=245) - In older children, Hirschsprung disease can be missed and typically presents with failure to thrive (small for age) and significant constipation. — Nelson Rosen (clinical) [Ep 15 · 4:46](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=286) - Children with Hirschsprung disease very rarely thrive and develop normally. — Nelson Rosen (clinical) [Ep 15 · 5:00](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=300) - In older children with suspected Hirschsprung disease, the diagnostic pathway includes plain films to assess degree of constipation, followed by contrast rectal enema, and then biopsy depending on findings. — Nelson Rosen (clinical) [Ep 15 · 5:20](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=320) - In older children with low-risk presentations (normal early years, then worsening constipation), biopsy should usually be done only after routine constipation management measures are tried and fail. — Nelson Rosen (clinical) [Ep 15 · 5:45](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=345) - Anorectal manometry uses a balloon catheter to measure pressures in the anus and rectum, specifically looking for the rectoanal inhibitory reflex. — Nelson Rosen (clinical) [Ep 15 · 6:25](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=385) - The normal rectoanal inhibitory reflex causes the anal sphincter to relax when a balloon is inflated in the rectum, but this reflex is absent in Hirschsprung disease. — Nelson Rosen (clinical) [Ep 15 · 7:05](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=425) - Anorectal manometry is not sufficiently sensitive to rule out Hirschsprung disease; a normal manometry does not completely exclude the diagnosis. — Nelson Rosen (clinical) [Ep 15 · 7:33](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=453) - Suction rectal biopsy can be performed at the bedside in newborns and is reliable up to one year of age. — Patty Curran (clinical) [Ep 15 · 8:20](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=500) - After one year of age, biopsy should be performed in the operating room under anesthesia to obtain tissue from higher in the rectum. — Patty Curran (clinical) [Ep 15 · 8:35](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=515) - For exam under anesthesia in Hirschsprung's patients, digital exam should assess for circumferential stricture at the anastomosis — Hira Ahmad (clinical) [Ep 11 · 1:36](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=96) - Hagar dilator can be used to size the anastomotic opening to ensure adequate caliber — Hira Ahmad (clinical) [Ep 11 · 1:57](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=117) - Foley catheter passage can determine if there is a twist in the pull-through segment — Hira Ahmad (clinical) [Ep 11 · 2:04](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=124) - Swabe cuff presents as a 1-2 centimeter circumferential narrowing that is not the anastomosis itself — Hira Ahmad (clinical) [Ep 11 · 2:23](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=143) - For patients with previous Duhamel procedure, full-thickness rectal biopsy should be performed posteriorly because that segment should be ganglionic — Hira Ahmad (clinical) [Ep 11 · 2:33](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=153) - In Duhamel procedure, rectal exam should assess for two lumens and a spur between them, as stool can flow into the Duhamel pouch, fill it, and compress the ganglionated pull-through — Marc Levitt (clinical) [Ep 11 · 3:00](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=180) - Treatment for problematic Duhamel spur is to take out the common wall; occasionally the Duhamel pouch itself needs excision — Marc Levitt (clinical) [Ep 11 · 3:25](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=205) - Before anesthesia induction, it is important to examine the anus for sphincteric contraction vs. patulous appearance — Marc Levitt (clinical) [Ep 11 · 3:36](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=216) - A patulous anus will not develop enterocolitis — Marc Levitt (clinical) [Ep 11 · 3:48](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=228) - During exam and biopsy, close examination is needed to ensure the dentate line was preserved at the original pull-through — Marc Levitt (clinical) [Ep 11 · 3:57](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=237) - If the dentate line has been lost at original pull-through or sphincters have been overstretched, the patient will not have enterocolitis but will have fecal incontinence — Marc Levitt (clinical) [Ep 11 · 4:12](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=252) - Botox is being studied to prevent enterocolitis with the theory of chemo-denervating nerves in the area instead of permanent stretch or anatomic destruction from surgery — Jason Frischer (clinical) [Ep 11 · 4:26](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=266) - Lone Star retractor should be used to obtain a circumferential view of the anastomotic area during exam under anesthesia — Rebecca Rentea (clinical) [Ep 11 · 4:57](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=297) - To palpate for Swabe cuff, place finger against the sacrum and pull down to feel rubbery tissue outside the pull-through — Marc Levitt (clinical) [Ep 11 · 14:08](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=848) - If repeat biopsy after pull-through shows no ganglion cells, it is very likely an aganglionotic or transition zone pull-through, though sampling error must be considered — Marc Levitt (clinical) [Ep 11 · 8:08](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=488) - Ganglion cells with hypertrophic nerves on repeat biopsy is more controversial; some surgeons offer redo for significantly obstructive patients with this finding — Marc Levitt (opinion) [Ep 11 · 8:33](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=513) - Hypertrophic nerves in the presence of ganglion cells can occur in functional constipation patients, and may develop in Hirschsprung's patients not emptying well due to sphincter dysfunction — Marc Levitt (clinical) [Ep 11 · 8:52](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=532) - Until comparing original and repeat pathology, it is unclear whether hypertrophic nerves represent original pathology error or secondary changes that evolved over time — Marc Levitt (clinical) [Ep 11 · 9:46](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=586) - The Swenson procedure was the first Hirschsprung's operation and is the purest, leaving the least amount of Hirschsprung's tissue behind — Marc Levitt (clinical) [Ep 11 · 13:05](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=785) - The Swabe and Duhamel procedures were developed because surgeons were doing the Swenson in too wide a plane and injuring the nervi erigentes in the mesorectum — Marc Levitt (clinical) [Ep 11 · 13:15](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=795) - If you stay right on the bowel wall during Swenson dissection, you avoid nerve injury — Marc Levitt (clinical) [Ep 11 · 13:24](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=804) - In the original laparoscopic Swabe described by Keith Jorgeson, a 5 centimeter cuff was recommended, which is too long — Marc Levitt (opinion) [Ep 11 · 12:40](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=760) - Many Swabe surgeons are now approaching a Swenson technique or have transitioned to Swenson, using a mini-cuff (approximately 1 centimeter) — Marc Levitt (clinical) [Ep 11 · 12:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=750) - Sometimes the Swabe cuff fuses back together or is not cut all the way, creating an aganglionotic obstructive ring around the pull-through — Marc Levitt (clinical) [Ep 11 · 13:45](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=825) - For redo pull-through in aganglionotic cases, the approach is total body prep, then transanal dissection in prone position first, going as far as possible; if healthy bowel cannot reach, be prepared for laparoscopy or laparotomy — Marc Levitt (clinical) [Ep 11 · 15:54](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=954) - Redo pull-throughs are easier to perform in prone position — Marc Levitt (opinion) [Ep 11 · 16:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=967) - Initial treatment for obstructed Hirschsprung's patient must include rectal irrigation, done early — Jason Frischer (guideline) [Ep 11 · 17:23](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=1043) - Every institution should have a protocol for obstructed Hirschsprung's patients to ensure quick and efficient response — Jason Frischer (guideline) [Ep 11 · 17:28](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=1048) - Systematic workup for obstructed Hirschsprung's includes: resuscitation, imaging studies, and comprehensive rectal exam under anesthesia — Jason Frischer (guideline) [Ep 11 · 17:41](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=1061) - Gastroenterologists and surgeons must know the anatomy of the original pull-through procedure (Swabe, Swenson, or Duhamel) when evaluating obstructed patients — Marc Levitt (guideline) [Ep 11 · 18:32](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-20-hirschsprung-disease-obstruction-part-2-4461?t=1112) - There are two types of problematic post-pull-through Hirschsprung's patients: obstructed patients (not emptying, distention, enterocolitis, failure to thrive) and soiling patients (pooping constantly, never distended, minimal constipation). — Marc Levitt (clinical) [Ep 12 · 1:41](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=101) - If the pull-through is done correctly with no anatomic problems and preservation of the sphincter mechanism, most Hirschsprung's patients do extremely well, though some need medical treatment to manage constipation. — Marc Levitt (clinical) [Ep 12 · 4:09](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=249) - There is no reason why any Hirschsprung patient should be obstructed or soiling if properly managed; if they are, investigation is needed to identify and fix the problem. — Marc Levitt (clinical) [Ep 12 · 4:40](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=280) - Pathology can change over time; cases exist where ganglion cells were present at pull-through but absent with hypertrophic nerves 7 years later when problems developed. — Jason Frischer (clinical) [Ep 12 · 5:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=307) - A pull-through can decompensate if the patient does not have adequate medical management or sphincter management, potentially leading to nerve hypertrophy. — Marc Levitt (clinical) [Ep 12 · 5:47](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=347) - The most important question for any soiling patient is: what is the patient's potential for bowel control? This question is often neglected. — Marc Levitt (clinical) [Ep 12 · 6:47](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=407) - All Hirschsprung's patients have the best possible potential for bowel control because they were born with normal sphincters (sometimes too strong and non-relaxing, but not lax) and intact dentate line with preserved anal canal sensation. — Marc Levitt (clinical) [Ep 12 · 7:09](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=429) - When a Hirschsprung's patient has an intact dentate line and intact sphincters, they have full potential for voluntary bowel movements and bowel control. — Marc Levitt (clinical) [Ep 12 · 7:38](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=458) - If the dentate line is lost (because dissection was started too low) or sphincters were overstretched and don't contract well, the patient may have lost their potential for bowel control. — Marc Levitt (clinical) [Ep 12 · 7:52](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=472) - 3D anorectal manometry can objectively assess whether sphincters are intact, whether the patient has a good squeeze, and whether that squeeze is concentric. — Marc Levitt (clinical) [Ep 12 · 9:15](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=555) - Sphincters should be assessed with the patient awake; examination under anesthesia makes it harder to assess sphincter function. — Jason Frischer (clinical) [Ep 12 · 9:58](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=598) - Sphincters become overstretched from transanal approach with deep dissection, wrong plane, or retractors placed in the anus; overstretched sphincters will not return to normal. — Marc Levitt (clinical) [Ep 12 · 10:48](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=648) - Patients with poor potential for bowel control (like anorectal malformation with poor spine/sacrum or spina bifida) need to be in a dedicated bowel management program with enemas or antegrade options. — Hira Ahmad (clinical) [Ep 12 · 12:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=740) - Patients with lost potential for bowel control (injured sphincters or lost dentate line) need a mechanical emptying program. — Marc Levitt (clinical) [Ep 12 · 12:39](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=759) - A mechanical program can get borderline patients clean and psychologically motivated to be clean, making them more likely to successfully potty train. — Marc Levitt (clinical) [Ep 12 · 12:53](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=773) - Mark Levitt's current routine is to perform 3D anorectal manometry in all soiling Hirschsprung's patients to assess squeeze quality and dentate line presence, then decide whether to attempt potty training with laxatives or start mechanical cleaning. — Marc Levitt (clinical) [Ep 12 · 13:11](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=791) - A new sphincter tightening technique has been developed and published in JPS with great results in multiple patients with disrupted or patulous sphincters. — Marc Levitt (clinical) [Ep 12 · 14:02](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=842) - Rectal prolapse after Hirschsprung's pull-through is iatrogenic and should never occur if sphincters are preserved; it results from overstretched sphincters becoming patulous to the point of laxity. — Marc Levitt (clinical) [Ep 12 · 15:02](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=902) - During normal bowel movements, sphincters relax and the anus opens with some mucosal descent, but as soon as sphincters tighten, the mucosa retracts back in; prolapse at rest indicates damaged muscles and/or mucosa brought too low at anastomosis. — Jason Frischer (clinical) [Ep 12 · 16:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=967) - For patients with patulous sphincters, Mark Levitt would offer sphincter reconstruction and perform a Malone at the same time, as the Malone can serve as a bridge to continence by allowing patients to practice holding and releasing flushes on command. — Marc Levitt (clinical) [Ep 12 · 17:13](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-22-hirschsprung-disease-the-soiling-patient-part-1-4616?t=1033) - The three components of continence are quality of sphincters, quality of dentate line, and motility. (clinical) [Ep 13 · 1:14](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=74) - In Hirschsprung's disease there are two sphincters of concern: the external sphincter (which patients have voluntary control of) and the internal sphincter (which tends not to relax due to absent recto-anal inhibitory reflex). (clinical) [Ep 13 · 1:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=90) - If a patient has voluntary bowel movements during the day but accidents at night when sleeping, their external sphincters are working but internal sphincters are not—when sleeping they relax the external sphincter and lose control. — Hira Ahmad (clinical) [Ep 13 · 2:12](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=132) - The dentate (or pectinate) line is the transition from squamous epithelium to columnar epithelium, occurring about two-thirds of the way up the anal canal, with associated changes in blood supply (splenic versus systemic) and innervation. (clinical) [Ep 13 · 2:44](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=164) - The nerves in the dentate line region tell you gas versus liquid versus solid, how hard to squeeze, how long to squeeze, and how tight to squeeze—preserving this region is key to continence. (clinical) [Ep 13 · 4:00](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=240) - The rectum (not the anal canal) has proprioception capacity to detect stretch, which signals stool accumulation and triggers external sphincter contraction to hold stool until a bathroom is found. (clinical) [Ep 13 · 4:58](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=298) - In anorectal malformation patients, stool softeners are problematic because patients never feel the stretch—they just have loose stool flowing; they are better off with bulk kicked out by a laxative than a stool softener that slowly oozes out. (clinical) [Ep 13 · 5:40](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=340) - For children with anorectal malformation, Hirschsprung's disease, or spinal conditions, their ability to sense stool in the rectum or neorectum is so sensitive to success that the right consistency and bulk of stool is very important. (clinical) [Ep 13 · 6:10](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=370) - If clinicians make stool too soft or too loose through medications, they throw a child with borderline control over the edge into failure. (clinical) [Ep 13 · 6:50](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=410) - Loose stool is the enemy of borderline continence because you don't know for sure that it's there—we are very dependent on the stretch and bulk of stool in the rectum to trigger the external sphincter and relax the internal sphincter. (clinical) [Ep 13 · 7:12](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=432) - Patients with Hirschsprung's disease with absolutely intact sphincters are dependent on rectal stretch; they don't have a rectum (it's been removed) and their sigmoid has taken over that job—if they have injured sphincters they are particularly in trouble. (clinical) [Ep 13 · 8:10](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=490) - A patient with missing dentate line (from dissection started too low) loses all anal canal sensation but can still develop bowel control if sphincters are working, though they will be very sensitive to loose stool and need bulk to detect stool presence. (clinical) [Ep 13 · 10:30](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=630) - A patient with missing dentate line but intact sphincters is similar to an anorectal malformation anastomosis (rectum or colon mucosa to skin) and should be able to achieve continence if muscle is intact. (clinical) [Ep 13 · 11:16](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=676) - On contrast study, a colon that moves too fast shows smaller caliber with many haustrations (appearing as contractions), while a colon that moves too slow shows dilation with lack of haustrations. — Hira Ahmad (clinical) [Ep 13 · 13:55](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=835) - For a hypomotile (slow-moving) colon with intact sphincters and dentate line, treatment can start with mechanical emptying then switch to laxative program; if sphincters and dentate line are deficient, mechanical enema program is needed. — Hira Ahmad (clinical) [Ep 13 · 15:41](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=941) - For hypermotile patients (7-8 stools daily), treatment involves constipating them and then ensuring timed emptying—either spontaneous if sphincters intact or mechanical if deficient. — Hira Ahmad (clinical) [Ep 13 · 17:45](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1065) - The treatment approach for hypermotile patients is to constipate them, then figure out how to empty them in a time-controlled fashion to maintain mechanical or social continence, depending on sphincter function. (clinical) [Ep 13 · 18:16](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1096) - Hirschsprung's is an obstruction problem that has been solved by surgery; the separate challenge is getting patients clean, which depends on whether they are too slow or too fast and whether they have the mechanisms for continence (sphincters and dentate line). (clinical) [Ep 13 · 19:08](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1148) - For hypermotile patients, treatment escalates through: skin care with cyanoacrylate barrier, proton pump inhibitor to reduce stool acidity, small volume enemas, water-soluble fiber for bulk, loperamide (0.5-0.8 mg/kg divided daily), cholestyramine, hyoscyamine (0.125 mg every 6 hours), and rarely diphenoxylate-atropine (which has cardiac side effects). (clinical) [Ep 13 · 19:49](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1189) - Tincture of opium is useful for slowing hypermotility but is a controlled substance and difficult to prescribe. (clinical) [Ep 13 · 21:54](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1314) - Botox may be needed for patients with good pull-through who are not emptying, to help them train and control non-relaxing sphincters and stop being withholders. (clinical) [Ep 13 · 22:06](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1326) - Some Hirschsprung's patients with good operations have super-strong sphincters that need relaxation (via Botox) to allow stool passage until they learn proper external and internal sphincter coordination for evacuation. (clinical) [Ep 13 · 22:34](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1354) - Anorectal manometry can provide objective data showing that resting pressures in some Hirschsprung's patients are on the higher end of normal, indicating need for relaxation therapy. (clinical) [Ep 13 · 23:10](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1390) - Patients with Hirschsprung's disease are very sensitive to some foods, particularly lactose, and paying attention to diet (from breastfed infants through older children) is important. (clinical) [Ep 13 · 23:40](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1420) - Every soiling patient can be made to do well with appropriate help (mechanical evacuations, Botox, etc.) and can be gotten on the right track. (opinion) [Ep 13 · 24:36](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1476) - Of all soiling populations (anorectal malformation, Hirschsprung's, functional constipation, and spinal), Hirschsprung's is the hardest group because the sphincters are so troublesome. (opinion) [Ep 13 · 25:10](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1510) - Within Hirschsprung's soiling patients, the hypermotile group is much harder to manage than the hypomotile group. (opinion) [Ep 13 · 25:20](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=1520) - Hirschsprung disease is a congenital anomaly of the GI tract that results in a functional bowel obstruction. — Jason Frischer (clinical) [Ep 2 · 0:33](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=33) - In Hirschsprung disease, the ganglion cells don't make it all the way down distally, and the colon ends up not being able to contract. — Aaron Garrison (clinical) [Ep 2 · 0:48](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=48) - More than 95% of neonates pass meconium within the first 48 hours of life. — Jason Frischer (clinical) [Ep 2 · 1:11](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=71) - Failure to pass meconium within the first 48 hours of life is typical of Hirschsprung's disease. — Jason Frischer (clinical) [Ep 2 · 1:11](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=71) - Hirschsprung disease has an incidence of about 1 in 5,000 children. — Aaron Garrison (epidemiological) [Ep 2 · 1:32](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=92) - About 10% of children with Hirschsprung disease will have a positive family history. — Jason Frischer (epidemiological) [Ep 2 · 1:43](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=103) - The RET gene is a predisposing genetic condition associated with Hirschsprung disease. — Jason Frischer (clinical) [Ep 2 · 1:43](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=103) - Up to 10% of children with Hirschsprung's disease will have trisomy 21. — Jason Frischer (epidemiological) [Ep 2 · 1:43](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=103) - Only 1 to 2% of patients with trisomy 21 have Hirschsprung's disease. — Jason Frischer (epidemiological) [Ep 2 · 1:43](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=103) - Hirschsprung disease is associated with Wordenberg syndrome and congenital central hyperventilation (Andine's curse). — Jason Frischer (clinical) [Ep 2 · 1:43](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=103) - The three studies that should be considered for evaluating for Hirschsprung's disease include contrast enema, rectal biopsy, and possibly anorectal manometry. — Jason Frischer (guideline) [Ep 2 · 2:25](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=145) - The classic finding on contrast enema is a transition zone in the rectosigmoid, but that transition zone can be located anywhere within the bowel. — Jason Frischer (clinical) [Ep 2 · 2:42](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=162) - The transition zone is from contracted rectum (where nerve cells are absent) to dilated rectum (where normal nerve cells are present). — Aaron Garrison (clinical) [Ep 2 · 2:55](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=175) - A rectosigmoid ratio of less than 1.0 is suggestive of Hirschsprung's disease. — Jason Frischer (clinical) [Ep 2 · 3:14](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=194) - In Hirschsprung's disease, the rectum is usually less dilated than the proximal colon, making the rectosigmoid ratio less than 1. — Aaron Garrison (clinical) [Ep 2 · 3:23](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=203) - Inability to evacuate contrast is a finding suggestive of Hirschsprung disease. — Aaron Garrison (clinical) [Ep 2 · 3:23](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=203) - In total colonic Hirschsprung's disease, a foreshortened or question mark colon can be seen on contrast enema. — Aaron Garrison (clinical) [Ep 2 · 3:23](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=203) - Rectal biopsy is the true definitive diagnosis for Hirschsprung disease. — Jason Frischer (guideline) [Ep 2 · 3:49](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=229) - Typical features on rectal biopsy include absence of ganglion cells, presence of hypertrophic nerves, abnormal pattern of colon esterase staining, and an absence of calretinine staining. — Jason Frischer (clinical) [Ep 2 · 4:04](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=244) - To be considered an adequate rectal biopsy, it must be taken from the rectum at least 1 cm above the dentate line and must include both mucosa and submucosal layers. — Jason Frischer (guideline) [Ep 2 · 4:04](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=244) - Suction rectal biopsy technique is typically used for patients less than 6 months of age. — Jason Frischer (guideline) [Ep 2 · 4:29](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=269) - Full thickness rectal biopsy technique should be considered for patients older than 6 months or when a suction biopsy is inadequate. — Jason Frischer (guideline) [Ep 2 · 4:29](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=269) - Suction rectal biopsy is painless and commonly done at the bedside in neonates. — Aaron Garrison (clinical) [Ep 2 · 4:47](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=287) - In anorectal manometry for Hirschsprung disease, there is a lack of the recto-anal inhibitory reflex (RAIR). — Aaron Garrison (clinical) [Ep 2 · 4:47](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=287) - The recto-anal inhibitory reflex may be absent in other conditions besides Hirschsprung disease, and some children have a false positive test. — Aaron Garrison (clinical) [Ep 2 · 4:47](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=287) - Any patient with an absent RAIR must undergo a rectal biopsy for confirmation of the diagnosis. — Jason Frischer (guideline) [Ep 2 · 5:42](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=342) - Children with Hirschsprung disease will need this condition managed for life, but the expectation is that they will live a normal life with close management and care. — Aaron Garrison (opinion) [Ep 2 · 6:07](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=367) - NPO babies diagnosed with Hirschsprung disease are typically managed with irrigations, antibiotics if they show evidence of enterocolitis, and NPO or NG tubes if they're distended. — Aaron Garrison (guideline) [Ep 2 · 6:37](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=397) - Older children with Hirschsprung disease are not amenable to just doing a primary pull through in many cases because the colon has become dilated. — Aaron Garrison (clinical) [Ep 2 · 7:03](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=423) - Older children with Hirschsprung disease will start on an enema program, and some may need diversion more proximally to give the colon time to decompress. — Aaron Garrison (guideline) [Ep 2 · 7:03](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=423) - The three goals of surgical management of Hirschsprung disease are: identify the extent of the aganglionic segment, resect that segment, and restore the bowel to its continuity. — Jason Frischer (guideline) [Ep 2 · 7:29](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=449) - The three procedures for Hirschsprung disease (Swensen, Suave, Duamel) all involve a transanal approach of removing the aganglionic colon and pulling down healthy colon and sewing it to the anus. — Jason Frischer (clinical) [Ep 2 · 7:59](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=479) - The Swensen technique is a full thickness dissection and anastomosis. — Jason Frischer (clinical) [Ep 2 · 7:59](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=479) - The Suave procedure is a mucosectomy where you leave a cuff of aganglionic bowel and bring the ganglionated bowel through that cuff of rectum and perform the anastomosis. — Jason Frischer (clinical) [Ep 2 · 7:59](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=479) - The Duamel procedure involves performing a pouch with an anastomosis of aganglionic and ganglionated bowel. — Jason Frischer (clinical) [Ep 2 · 7:59](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=479) - Early postoperative complications include diaper rash and excoriation that can often need to be treated like a burn. — Aaron Garrison (clinical) [Ep 2 · 8:48](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=528) - Anastomotic leak is a rare but possible early complication after Hirschsprung surgery. — Aaron Garrison (clinical) [Ep 2 · 8:48](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=528) - Hirschsprung's associated enterocolitis is the main early complication everyone needs to be aware of. — Aaron Garrison (opinion) [Ep 2 · 8:48](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=528) - Hirschsprung's associated enterocolitis is poorly understood and likely is an inflammatory condition secondary to bacterial overgrowth. — Jason Frischer (clinical) [Ep 2 · 9:15](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=555) - A child with enterocolitis presents with abdominal distention, vomiting, fever, and change in bowel habits. — Jason Frischer (clinical) [Ep 2 · 9:15](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=555) - Enterocolitis must be recognized as potential enterocolitis and treated urgently. — Jason Frischer (guideline) [Ep 2 · 9:15](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=555) - Treatment for enterocolitis includes fluid resuscitation, digital rectal exam, and colonic irrigations. — Jason Frischer (guideline) [Ep 2 · 9:15](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=555) - Depending on the severity of enterocolitis, broad-spectrum antibiotics are sometimes added, and patients are usually started on metronidazole. — Jason Frischer (guideline) [Ep 2 · 9:15](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=555) - About 80% of kids with Hirschsprung's disease are constipated and will need some kind of management. — Aaron Garrison (epidemiological) [Ep 2 · 10:09](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=609) - Assuming the operation has been done well and there are no transition zone or strictures, most patients with Hirschsprung disease are expected to do very well and be in kindergarten socially confident. — Aaron Garrison (opinion) [Ep 2 · 10:09](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=609) - 90% of Hirschsprung disease is diagnosed in the first month of life — Levitt (epidemiological) [Ep 14 · 3:50](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=230) - Of 1000 constipated patients, approximately 900 can be managed by pediatrician with diet and laxatives, 90 of the remaining 100 by gastroenterologist, leaving 10 requiring surgical evaluation and 1 needing surgical intervention — Levitt (epidemiological) [Ep 14 · 2:02](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=122) - Anorectal manometry is not reliable under age one year, though many gastroenterologists disagree with this assessment — Levitt (opinion) [Ep 14 · 6:37](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=397) - Suction rectal biopsy is reliable under 6 months of age, gray zone between 6-12 months depending on baby size, and over 10-12 months should transition to open biopsy in OR — Caitlin Smith (clinical) [Ep 14 · 9:32](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=572) - Hirschsprung diagnosis requires both absence of ganglion cells on 100 levels AND presence of hypertrophic nerves; do not operate on absence of ganglion cells alone — Levitt (clinical) [Ep 14 · 10:36](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=636) - Hypertrophic nerves are defined as greater than 40 microns, a standard defined by Margaret Collins — Levitt (clinical) [Ep 14 · 11:04](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=664) - If ganglion cells are present, it is not Hirschsprung disease regardless of whether hypertrophic nerves are present; hypertrophic nerves in this context are secondary to constipation — Levitt (clinical) [Ep 14 · 12:23](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=743) - Helen Noblett, a pediatric surgeon in Melbourne, Australia, developed the suction rectal biopsy gun and was the only woman among early Hirschsprung surgery icons — Levitt (clinical) [Ep 14 · 8:16](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=496) - For profuse rectal bleeding after suction biopsy, management is digital rectal insertion with direct pressure against the sacrum to tamponade bleeding from the posterior rectal wall — Caitlin Smith (clinical) [Ep 14 · 8:59](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=539) - In the recto-anal inhibitory reflex (RARE), smooth muscle normally relaxes when rectum is distended; in Hirschsprung disease and internal sphincter achalasia, the muscle contracts instead — Levitt (clinical) [Ep 14 · 5:05](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=305) - Coordinated OR approach: perform anorectal manometry awake with anesthesia standby, if RARE absent then proceed with biopsy, and consider Botox injection for potential internal sphincter achalasia treatment — Levitt (clinical) [Ep 14 · 5:48](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=348) - Older children with Hirschsprung typically have very short segment aganglionosis and their proximal colon can overcome the aganglionic segment most of the time, which is how they get by until diagnosis — Fisher (clinical) [Ep 14 · 13:16](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=796) - For severely distended colon in delayed Hirschsprung, options include aggressive irrigations for 1-2 months, leveling colostomy, or ileostomy; preference is for irrigations first, then ileostomy if diversion needed rather than leveling colostomy — Levitt (clinical) [Ep 14 · 15:10](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=910) - Tapered colon segments do not function well; preference is to decompress with stoma, wait for colon to shrink, then perform pull-through to normal caliber proximal sigmoid or left colon — Levitt (opinion) [Ep 14 · 16:35](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=995) - Technique for anastomosing slightly larger circle to slightly smaller circle: place stitches at 12, 6, 3, and 9 o'clock positions, then continue dividing between those stitches — Levitt (clinical) [Ep 14 · 17:06](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=1026) - Laparoscopic approach is safer than transanal-only for obtaining true level confirmation with full-thickness biopsy and performing mesenteric dissection, thereby decreasing transanal dissection time and reducing sphincter injury risk — Fisher (opinion) [Ep 14 · 18:25](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=1105) - Transanal-only dissection might be appropriate in older children with rectal transition zone where level is very confident and operation can be performed at anal level without sphincter stretching, preferably in prone position — Levitt (opinion) [Ep 14 · 19:21](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=1161) - Rectal biopsy showing squamous epithelium indicates inadequate specimen that is too distal — Caitlin Smith (clinical) [Ep 14 · 11:32](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=692) - Rectal biopsy specimen filled with eosinophils suggests milk protein allergy, which can mimic Hirschsprung appearance but is ruled out by presence of ganglion cells — Levitt (clinical) [Ep 14 · 12:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=720) - Suction rectal biopsies are taken at 1 centimeter, 2 centimeters, and 3 centimeters from the anal verge using the suction biopsy gun — Caitlin Smith (clinical) [Ep 14 · 8:03](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=483) - Adequate rectal biopsy specimen must contain sufficient submucosa to determine specimen adequacy — Caitlin Smith (clinical) [Ep 14 · 10:38](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=638) - Contrast enema in Hirschsprung should show rectum as widest part of colon; inverted ratio with sigmoid wider than rectum suggests Hirschsprung — Caitlin Smith (clinical) [Ep 14 · 12:53](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=773) - Red flags for Hirschsprung referral include marginal weight gain and requirement for rectal stimulation to produce any bowel movement — Caitlin Smith (clinical) [Ep 14 · 3:09](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=189) - Older children with delayed Hirschsprung diagnosis typically never develop enterocolitis despite years of symptoms — Levitt (clinical) [Ep 14 · 3:58](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=238) - Anorectal manometry requires awake, cooperative patient to obtain accurate resting pressure for external sphincter and pelvic floor information; sedated patients will not have resting pressure — Levitt (clinical) [Ep 14 · 6:56](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=416) - Dyssynergia (sphincter not working in coordination with body) is often seen in 3-year-old age group during potty training and can be detected on anorectal manometry — Fisher (clinical) [Ep 14 · 7:14](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=434) - Rectal irrigations should be taught to families when moderate concern for Hirschsprung exists but biopsy is refused, as potential preventive measure — Fisher (opinion) [Ep 14 · 14:08](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=848) - Patient underwent primary laparoscopic-assisted Swenson procedure, recovered well postoperatively with 5-day hospital stay, and is currently stooling well with flatter abdomen — Caitlin Smith (clinical) [Ep 14 · 19:43](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=1183) ## 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 4 · 1:59](https://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 4 · 2:44](https://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 4 · 3:10](https://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 4 · 4:28](https://library.globalcastmd.com/watch/hirschsprung-disease-part-i-with-marc-levitt-311?t=268) - The 3-millimeter sealer is used as the primary mode of dissection for laparoscopic-assisted pull-through in Hirschsprung's disease. — The host summarizing the discussion [Ep 16 · 0:00](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=0) - The patient is a newborn weighing 3.2 kg. — The host summarizing the discussion [Ep 16 · 0:32](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=32) - A supraumbilical ring incision is used for the Veress needle and 4-millimeter trocar camera port in small newborns, with the trocar placed just to the left of midline to avoid the umbilical vein. — The host summarizing the discussion [Ep 16 · 0:36](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=36) - A full-thickness biopsy is obtained from the muscular wall approximately 5 centimeters above the transition zone between the distal sigmoid and rectum. — The host summarizing the discussion [Ep 16 · 0:53](https://library.globalcastmd.com/watch/technique-laparoscopic-assisted-pull-through-for-hirschsprung-s-disease-756?t=53) - A one-day-old full-term infant weighing 3.9 kilograms presented with significant abdominal distension and bilious emesis. — Todd Ponsky summarizing the discussion [Ep 6 · 0:40](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=40) - If a child is sick with bilious emesis and distension, resuscitation should be the first step before diagnostic workup. — Rod Gerardo summarizing the discussion [Ep 6 · 2:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-3-5-proximal-hirschsprung-disease-3682?t=127) - The reinforcement layer of sutures is critical for lining up the two pieces of bowel to achieve mucosa-to-mucosa edge approximation. — Rod Gerardo summarizing the discussion [Ep 7 · 17:01](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-4-classic-hirschsprung-disease-surgical-technique-3704?t=1021) - Frozen sections can rule out Hirschsprung disease but cannot definitively rule it in. — Rod Gerardo summarizing the discussion [Ep 8 · 5:23](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=323) - For proximal disease, the key question on biopsy is whether ganglion cells are present, not whether nerves are hypertrophic. — Rod Gerardo summarizing the discussion [Ep 8 · 6:00](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-5-proximal-hirschsprung-disease-surgical-technique-3745?t=360) - After meconium passage, obtaining another contrast image will show the characteristic appearance of Hirschsprung disease — Amanda Jensen summarizing the discussion [Ep 9 · 3:53](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=233) - Common causes of failure to pass meconium include Hirschsprung disease, meconium plug syndrome, meconium ileus, and anorectal malformation — Rod Gerardo summarizing the discussion [Ep 9 · 4:07](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=247) - Rare causes of failure to pass meconium include small left colon syndrome, hypothyroidism, opiates in the newborn system, magnesium sulfate from maternal preterm labor treatment, milk protein allergy, and microcolon intestinal hypoperistalsis syndrome — Amanda Jensen summarizing the discussion [Ep 9 · 4:19](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=259) - Rectal biopsies should be attempted preoperatively before proceeding to the operating room — Amanda Jensen summarizing the discussion [Ep 9 · 8:03](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=483) - If the patient is not doing well with irrigations, a procedure such as ileostomy or leveling colostomy is necessary to relieve pressure — Amanda Jensen summarizing the discussion [Ep 9 · 8:53](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=533) - Loop ostomies should be avoided in Hirschsprung disease because stool will likely spill into the non-functional part of the colon — Amanda Jensen summarizing the discussion [Ep 9 · 9:04](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=544) - A transition zone can often be visualized during surgery (open or laparoscopic), but when it cannot be seen, biopsies should be obtained at multiple levels — Amanda Jensen summarizing the discussion [Ep 9 · 9:35](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=575) - It is acceptable to not perform a definitive procedure if only frozen section is available and there is uncertainty about the frozen section diagnosis — Amanda Jensen summarizing the discussion [Ep 9 · 10:09](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=609) - Surgeons can wait for final pathology and return another day to perform the pull-through procedure if needed — Amanda Jensen summarizing the discussion [Ep 9 · 10:21](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-10-total-colonic-hirschsprung-disease-part-1-3893?t=621) - Even with suggestive manometry findings, a biopsy is still required before surgical intervention; no surgeon would operate on manometry alone. — Rod Gerardo summarizing the discussion [Ep 15 · 7:50](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=470) - Normal individuals without Hirschsprung disease have no ganglion cells in the very first (distal-most) part of the rectum. — Rod Gerardo summarizing the discussion [Ep 15 · 8:41](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=521) - In children over one year old, the suction biopsy tool cannot obtain adequate tissue depth because the tissue is thicker, necessitating surgical biopsy in the OR. — Rod Gerardo summarizing the discussion [Ep 15 · 9:05](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=545) - Surgical rectal biopsy in older children is a simple 20-minute procedure with same-day discharge. — Rod Gerardo summarizing the discussion [Ep 15 · 9:40](https://library.globalcastmd.com/watch/hirschsprung-disease-workup-4411?t=580) - Nocturnal soiling with daytime control can occur if the dentate line is lost (from overstretching) with some preservation of the external sphincter. — Amanda Jensen summarizing the discussion [Ep 13 · 2:29](https://library.globalcastmd.com/watch/the-colorectal-quiz-episode-23-hirschsprung-disease-the-soiling-patient-part-2-the-dentate-line-and-motility-4739?t=149) - The first reports of Hirschsprung disease date back to the 17th century. — The host summarizing the discussion [Ep 2 · 0:00](https://library.globalcastmd.com/watch/hirschsprung-disease-in-brief-5023?t=0) - GI literature reports adequate suction rectal biopsy tissue obtained up to 6 years of age — Fisher summarizes what Dr. Caitlin Smith said [Ep 14 · 10:00](https://library.globalcastmd.com/watch/colorectal-quiz-episode-27-delayed-hirschsprung-disease-5052?t=600) --- Educational content from recorded physician discussions — not medical advice. 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