Most of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops
Two definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis
Most cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer
In Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)
Laparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch
clinicalFalse positive contrast enemas showing transition zones can occur in newborns without Hirschsprung disease↗
▶Ep 49 · 8:16
epidemiologicalIn Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)↗
▶Ep 49 · 10:31
quoteyou just have to get one patient who loses his endotracheal tube while you're doing that and it kind of sours you on that↗
▶Ep 49 · 14:33
clinicalManometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older children without need for biopsy↗
▶Ep 49 · 14:52
clinicalAbsence of recto-anal inhibitory reflex on manometry requires biopsy confirmation due to false positives↗
▶Ep 49 · 15:23
clinicalTwo definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis↗
▶Ep 49 · 15:23
quoteThere are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one↗
▶Ep 49 · 16:09
quoteit's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Why, you know, it's just he's, he's the big spectrum guy, right? There's a spectrum of disease, right?↗
▶Ep 49 · 16:36
opinionFor ultra-short segment with confirmed aganglionosis, pull-through is preferred over myectomy↗
▶Ep 49 · 16:57
clinicalMost 16-year-olds with newly diagnosed Hirschsprung require diversion due to massive bowel dilation↗
▶Ep 49 · 17:05
opinionLaparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch↗
▶Ep 49 · 17:05
quoteI find that, that rectum gets so thickened trying to do it transanally, uh, you get into the wrong plane, you end up stretching the sphincters way more than you want to↗
▶Ep 49 · 17:53
clinicalTransitional epithelium (not normal rectal mucosa) should be seen on biopsy if specimen is truly from anal canal↗
▶Ep 49 · 21:10
clinicalMost cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer↗
▶Ep 49 · 21:10
quoteMost of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops↗
▶Ep 49 · 21:10
opinionFor cecal perforation, close perforation and create loop ileostomy without frozen section of ileum, as total colonic disease is unlikely↗
▶Ep 49 · 22:52
clinicalIn long-segment disease, delay pull-through 6-12 months until stoma output thickens to prevent severe perianal excoriation↗
▶Ep 49 · 23:50
opinionPull-throughs using only cecum (very short colonic segment) have poor outcomes with stasis and enterocolitis; ileal Duhamel may be preferable↗
▶Ep 49 · 24:37
opinionIf transition zone is at hepatic flexure (not just cecum), preserve the colon and perform Duhamel↗
▶Ep 49 · 25:47
clinicalWhen bringing right colon down, flip it over rather than rotating; preserve marginal artery and let anatomy determine orientation↗
▶Ep 49 · 29:04
clinicalManometry is not reliably performed until age 5-6 years↗
▶Ep 49 · 30:17
opinionFor 3-year-old with massive dilation, divert and attempt bowel decompression; resect dilated segment if it does not collapse after 6-8 months↗
▶Ep 49 · 32:47
quoteI have had success in, uh, getting that distal bowel to, uh, shrink in size. With the stoma for 6 or 8 months↗
▶Ep 49 · 32:57
clinicalOlder children are less likely to have bowel shrinkage after diversion, but 3-year-olds may still respond↗
▶Ep 49 · 32:57
quotethe older the kid, the less likely that it's going to shrink down in size. But, uh, at this age I think it will↗
▶Ep 49 · 37:00
guidelineFor recurrent enterocolitis, first rule out distal obstruction and residual aganglionosis with exam under anesthesia and biopsies↗
▶Ep 49 · 37:32
clinicalChronic oral metronidazole is used liberally for recurrent enterocolitis; some patients require it for months, and symptoms recur when discontinued↗
▶Ep 49 · 37:39
quotewe have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. So, some of them need it for a long, long time↗
▶Ep 49 · 38:03
clinicalBotox injection decreases number of hospitalizations for enterocolitis, though it does not work in all patients↗
▶Ep 49 · 38:03
quotewe actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though↗
Journal of Pediatric Surgery Article Review: May 2023, CAPS Issue
▶Ep 144 · 2:24
clinicalThree risk factors for Hirschsprung-associated IBD are trisomy 21, a history of enterocolitis following pull-through surgery, and long segment disease.↗
▶Ep 144 · 2:24
quoteThe three things. That came up as risk factors, trisomy 21, a history of enterocolitis following Coulter's surgery, and long segment disease.↗
▶Ep 144 · 3:05
quoteThis Hirschsprung's associated IBD is very poorly defined, and it presents in a number of different ways, and we have no idea why it happens.↗
▶Ep 144 · 3:05
clinicalHirschsprung's associated IBD is very poorly defined, presents in a number of different ways, and the reason why it happens is unknown.↗
Jacob's statements about Enterocolitis30 statements
clinicalFalse positive contrast enemas showing transition zones can occur in newborns without Hirschsprung disease↗
▶Ep 11 · 8:16
epidemiologicalIn Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)↗
▶Ep 11 · 10:31
quoteyou just have to get one patient who loses his endotracheal tube while you're doing that and it kind of sours you on that↗
▶Ep 11 · 14:33
clinicalManometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older children without need for biopsy↗
▶Ep 11 · 14:52
clinicalAbsence of recto-anal inhibitory reflex on manometry requires biopsy confirmation due to false positives↗
▶Ep 11 · 15:23
clinicalTwo definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis↗
▶Ep 11 · 15:23
quoteThere are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one↗
▶Ep 11 · 16:09
quoteit's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Why, you know, it's just he's, he's the big spectrum guy, right? There's a spectrum of disease, right?↗
▶Ep 11 · 16:36
opinionFor ultra-short segment with confirmed aganglionosis, pull-through is preferred over myectomy↗
▶Ep 11 · 16:57
clinicalMost 16-year-olds with newly diagnosed Hirschsprung require diversion due to massive bowel dilation↗
▶Ep 11 · 17:05
quoteI find that, that rectum gets so thickened trying to do it transanally, uh, you get into the wrong plane, you end up stretching the sphincters way more than you want to↗
▶Ep 11 · 17:05
opinionLaparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch↗
▶Ep 11 · 17:53
clinicalTransitional epithelium (not normal rectal mucosa) should be seen on biopsy if specimen is truly from anal canal↗
▶Ep 11 · 21:10
quoteMost of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops↗
▶Ep 11 · 21:10
clinicalMost cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer↗
▶Ep 11 · 21:10
opinionFor cecal perforation, close perforation and create loop ileostomy without frozen section of ileum, as total colonic disease is unlikely↗
▶Ep 11 · 22:52
clinicalIn long-segment disease, delay pull-through 6-12 months until stoma output thickens to prevent severe perianal excoriation↗
▶Ep 11 · 23:50
opinionPull-throughs using only cecum (very short colonic segment) have poor outcomes with stasis and enterocolitis; ileal Duhamel may be preferable↗
▶Ep 11 · 24:37
opinionIf transition zone is at hepatic flexure (not just cecum), preserve the colon and perform Duhamel↗
▶Ep 11 · 25:47
clinicalWhen bringing right colon down, flip it over rather than rotating; preserve marginal artery and let anatomy determine orientation↗
▶Ep 11 · 29:04
clinicalManometry is not reliably performed until age 5-6 years↗
▶Ep 11 · 30:17
opinionFor 3-year-old with massive dilation, divert and attempt bowel decompression; resect dilated segment if it does not collapse after 6-8 months↗
▶Ep 11 · 32:47
quoteI have had success in, uh, getting that distal bowel to, uh, shrink in size. With the stoma for 6 or 8 months↗
▶Ep 11 · 32:57
clinicalOlder children are less likely to have bowel shrinkage after diversion, but 3-year-olds may still respond↗
▶Ep 11 · 32:57
quotethe older the kid, the less likely that it's going to shrink down in size. But, uh, at this age I think it will↗
▶Ep 11 · 37:00
guidelineFor recurrent enterocolitis, first rule out distal obstruction and residual aganglionosis with exam under anesthesia and biopsies↗
▶Ep 11 · 37:32
clinicalChronic oral metronidazole is used liberally for recurrent enterocolitis; some patients require it for months, and symptoms recur when discontinued↗
▶Ep 11 · 37:39
quotewe have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. So, some of them need it for a long, long time↗
▶Ep 11 · 38:03
clinicalBotox injection decreases number of hospitalizations for enterocolitis, though it does not work in all patients↗
▶Ep 11 · 38:03
quotewe actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though↗
Jacob's statements about Enterocolitis30 statements
clinicalFalse positive contrast enemas showing transition zones can occur in newborns without Hirschsprung disease↗
▶Ep 11 · 8:16
epidemiologicalIn Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)↗
▶Ep 11 · 10:31
quoteyou just have to get one patient who loses his endotracheal tube while you're doing that and it kind of sours you on that↗
▶Ep 11 · 14:33
clinicalManometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older children without need for biopsy↗
▶Ep 11 · 14:52
clinicalAbsence of recto-anal inhibitory reflex on manometry requires biopsy confirmation due to false positives↗
▶Ep 11 · 15:23
clinicalTwo definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis↗
▶Ep 11 · 15:23
quoteThere are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one↗
▶Ep 11 · 16:09
quoteit's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Why, you know, it's just he's, he's the big spectrum guy, right? There's a spectrum of disease, right?↗
▶Ep 11 · 16:36
opinionFor ultra-short segment with confirmed aganglionosis, pull-through is preferred over myectomy↗
▶Ep 11 · 16:57
clinicalMost 16-year-olds with newly diagnosed Hirschsprung require diversion due to massive bowel dilation↗
▶Ep 11 · 17:05
quoteI find that, that rectum gets so thickened trying to do it transanally, uh, you get into the wrong plane, you end up stretching the sphincters way more than you want to↗
▶Ep 11 · 17:05
opinionLaparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch↗
▶Ep 11 · 17:53
clinicalTransitional epithelium (not normal rectal mucosa) should be seen on biopsy if specimen is truly from anal canal↗
▶Ep 11 · 21:10
opinionFor cecal perforation, close perforation and create loop ileostomy without frozen section of ileum, as total colonic disease is unlikely↗
▶Ep 11 · 21:10
clinicalMost cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer↗
▶Ep 11 · 21:10
quoteMost of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops↗
▶Ep 11 · 22:52
clinicalIn long-segment disease, delay pull-through 6-12 months until stoma output thickens to prevent severe perianal excoriation↗
▶Ep 11 · 23:50
opinionPull-throughs using only cecum (very short colonic segment) have poor outcomes with stasis and enterocolitis; ileal Duhamel may be preferable↗
▶Ep 11 · 24:37
opinionIf transition zone is at hepatic flexure (not just cecum), preserve the colon and perform Duhamel↗
▶Ep 11 · 25:47
clinicalWhen bringing right colon down, flip it over rather than rotating; preserve marginal artery and let anatomy determine orientation↗
▶Ep 11 · 29:04
clinicalManometry is not reliably performed until age 5-6 years↗
▶Ep 11 · 30:17
opinionFor 3-year-old with massive dilation, divert and attempt bowel decompression; resect dilated segment if it does not collapse after 6-8 months↗
▶Ep 11 · 32:47
quoteI have had success in, uh, getting that distal bowel to, uh, shrink in size. With the stoma for 6 or 8 months↗
▶Ep 11 · 32:57
clinicalOlder children are less likely to have bowel shrinkage after diversion, but 3-year-olds may still respond↗
▶Ep 11 · 32:57
quotethe older the kid, the less likely that it's going to shrink down in size. But, uh, at this age I think it will↗
▶Ep 11 · 37:00
guidelineFor recurrent enterocolitis, first rule out distal obstruction and residual aganglionosis with exam under anesthesia and biopsies↗
▶Ep 11 · 37:32
clinicalChronic oral metronidazole is used liberally for recurrent enterocolitis; some patients require it for months, and symptoms recur when discontinued↗
▶Ep 11 · 37:39
quotewe have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. So, some of them need it for a long, long time↗
▶Ep 11 · 38:03
quotewe actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though↗
▶Ep 11 · 38:03
clinicalBotox injection decreases number of hospitalizations for enterocolitis, though it does not work in all patients↗
Jacob's statements about Hirschsprung disease34 statements
clinicalFalse positive contrast enemas showing transition zones can occur in newborns without Hirschsprung disease↗
▶Ep 24 · 8:16
epidemiologicalIn Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)↗
▶Ep 24 · 10:31
quoteyou just have to get one patient who loses his endotracheal tube while you're doing that and it kind of sours you on that↗
▶Ep 24 · 14:33
clinicalManometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older children without need for biopsy↗
▶Ep 24 · 14:52
clinicalAbsence of recto-anal inhibitory reflex on manometry requires biopsy confirmation due to false positives↗
▶Ep 24 · 15:23
clinicalTwo definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis↗
▶Ep 24 · 15:23
quoteThere are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one↗
▶Ep 24 · 16:09
quoteit's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Why, you know, it's just he's, he's the big spectrum guy, right? There's a spectrum of disease, right?↗
▶Ep 24 · 16:36
opinionFor ultra-short segment with confirmed aganglionosis, pull-through is preferred over myectomy↗
▶Ep 24 · 16:57
clinicalMost 16-year-olds with newly diagnosed Hirschsprung require diversion due to massive bowel dilation↗
▶Ep 24 · 17:05
quoteI find that, that rectum gets so thickened trying to do it transanally, uh, you get into the wrong plane, you end up stretching the sphincters way more than you want to↗
▶Ep 24 · 17:05
opinionLaparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch↗
▶Ep 24 · 17:53
clinicalTransitional epithelium (not normal rectal mucosa) should be seen on biopsy if specimen is truly from anal canal↗
▶Ep 24 · 21:10
opinionFor cecal perforation, close perforation and create loop ileostomy without frozen section of ileum, as total colonic disease is unlikely↗
▶Ep 24 · 21:10
clinicalMost cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer↗
▶Ep 24 · 21:10
quoteMost of these cecal perforations from Hirschberg's disease are not total colonic disease. Most of them are shorter segment disease. It's like with a rectal cancer. The cecum gets more distended and pops↗
▶Ep 24 · 22:52
clinicalIn long-segment disease, delay pull-through 6-12 months until stoma output thickens to prevent severe perianal excoriation↗
▶Ep 24 · 23:50
opinionPull-throughs using only cecum (very short colonic segment) have poor outcomes with stasis and enterocolitis; ileal Duhamel may be preferable↗
▶Ep 24 · 24:37
opinionIf transition zone is at hepatic flexure (not just cecum), preserve the colon and perform Duhamel↗
▶Ep 24 · 25:47
clinicalWhen bringing right colon down, flip it over rather than rotating; preserve marginal artery and let anatomy determine orientation↗
▶Ep 24 · 29:04
clinicalManometry is not reliably performed until age 5-6 years↗
▶Ep 24 · 30:17
opinionFor 3-year-old with massive dilation, divert and attempt bowel decompression; resect dilated segment if it does not collapse after 6-8 months↗
▶Ep 24 · 32:47
quoteI have had success in, uh, getting that distal bowel to, uh, shrink in size. With the stoma for 6 or 8 months↗
▶Ep 24 · 32:57
clinicalOlder children are less likely to have bowel shrinkage after diversion, but 3-year-olds may still respond↗
▶Ep 24 · 32:57
quotethe older the kid, the less likely that it's going to shrink down in size. But, uh, at this age I think it will↗
▶Ep 24 · 37:00
guidelineFor recurrent enterocolitis, first rule out distal obstruction and residual aganglionosis with exam under anesthesia and biopsies↗
▶Ep 24 · 37:32
clinicalChronic oral metronidazole is used liberally for recurrent enterocolitis; some patients require it for months, and symptoms recur when discontinued↗
▶Ep 24 · 37:39
quotewe have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. So, some of them need it for a long, long time↗
▶Ep 24 · 38:03
quotewe actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though↗
▶Ep 24 · 38:03
clinicalBotox injection decreases number of hospitalizations for enterocolitis, though it does not work in all patients↗
Journal of Pediatric Surgery Article Review: May 2023, CAPS Issue
▶Ep 60 · 2:24
clinicalThree risk factors for Hirschsprung-associated IBD are trisomy 21, a history of enterocolitis following pull-through surgery, and long segment disease.↗
▶Ep 60 · 2:24
quoteThe three things. That came up as risk factors, trisomy 21, a history of enterocolitis following Coulter's surgery, and long segment disease.↗
▶Ep 60 · 3:05
clinicalHirschsprung's associated IBD is very poorly defined, presents in a number of different ways, and the reason why it happens is unknown.↗
▶Ep 60 · 3:05
quoteThis Hirschsprung's associated IBD is very poorly defined, and it presents in a number of different ways, and we have no idea why it happens.↗
Jacob's statements about Inflammatory Bowel Disease4 statements
Journal of Pediatric Surgery Article Review: May 2023, CAPS Issue
▶Ep 4 · 2:24
clinicalThree risk factors for Hirschsprung-associated IBD are trisomy 21, a history of enterocolitis following pull-through surgery, and long segment disease.↗
▶Ep 4 · 2:24
quoteThe three things. That came up as risk factors, trisomy 21, a history of enterocolitis following Coulter's surgery, and long segment disease.↗
▶Ep 4 · 3:05
quoteThis Hirschsprung's associated IBD is very poorly defined, and it presents in a number of different ways, and we have no idea why it happens.↗
▶Ep 4 · 3:05
clinicalHirschsprung's associated IBD is very poorly defined, presents in a number of different ways, and the reason why it happens is unknown.↗
