On routine ultrasound, these findings, especially early on in the, you know, 20 week range when most ultrasounds are done, they may be very subtle, and there may be nothing that, that tips the community obstetrician off that there's anything wrong.
There are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis
Early papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit
Early papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit
For pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization
There are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 6 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 6 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 6 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 6 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 6 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 6 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 6 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 6 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 6 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 6 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 6 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 6 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 6 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 6 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 6 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 6 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 6 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 6 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 6 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 6 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 6 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 6 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 6 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 6 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 6 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 6 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 6 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 6 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 6 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 6 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 6 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 6 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 6 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 6 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 6 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 6 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 6 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 6 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 6 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 6 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 6 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 6 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 6 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 6 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 6 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 6 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 6 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 6 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 6 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 6 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 6 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 6 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 6 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 6 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 6 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 6 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 6 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 6 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 6 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 6 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 6 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 6 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 6 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 6 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 6 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 6 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 6 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 6 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 6 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 6 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 6 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 6 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 6 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 20 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 20 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 20 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 20 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 20 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 20 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 20 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 20 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 20 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 20 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 20 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 20 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 20 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 20 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 20 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 20 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 20 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 20 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 20 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 20 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 20 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 20 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 20 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 20 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 20 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 20 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 20 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 20 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 20 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 20 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 20 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 20 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 20 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 20 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 20 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 20 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 20 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 20 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 20 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 20 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 20 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 20 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 20 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 20 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 20 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 20 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 20 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 20 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 20 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 20 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 20 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 20 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 20 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 20 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 20 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 20 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 20 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 20 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 20 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 20 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 20 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 20 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 20 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 20 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 20 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 20 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 20 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 20 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 20 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 20 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 20 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 20 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 20 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 20 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 20 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 20 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 20 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 20 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 20 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 20 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 20 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 20 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 20 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 20 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 20 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 20 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 20 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 20 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 20 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 20 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 20 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 20 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 20 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 20 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 20 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 20 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 20 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 20 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 20 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 20 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 20 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 20 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 20 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 20 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 20 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 20 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 20 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 20 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 20 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 20 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 20 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 20 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 20 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 20 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 20 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 20 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 20 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 20 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 20 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 20 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 20 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 20 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 20 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 20 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 20 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 20 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 20 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 20 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 20 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 20 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 20 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 20 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 20 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 20 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 20 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 20 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 20 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 20 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 20 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 20 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 20 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 20 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 20 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 20 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 20 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 20 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 20 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 20 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 20 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 20 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 20 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 20 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 20 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 20 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 20 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 20 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 20 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 20 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 20 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 20 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 20 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 20 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 20 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 20 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 20 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 20 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 20 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
▶Ep 20 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
Jack's statements about Anorectal Malformation5 statements
clinicalAt 20 weeks gestation when most ultrasounds are done, cloacal findings may be very subtle with nothing that tips the community obstetrician off that there's anything wrong↗
▶Ep 10 · 35:16
quoteI think you, you have to have a, a high index of suspicion to start looking for these subtle findings and, and I just think most people out there in the, in the real world aren't even thinking about this possibility.↗
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
▶Ep 19 · 30:18
epidemiologicalAt Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.↗
▶Ep 19 · 30:26
opinionFindings of cloaca on routine 20-week ultrasound may be very subtle; community obstetricians may not suspect the diagnosis unless they have a high index of suspicion.↗
▶Ep 19 · 30:26
quoteOn routine ultrasound, these findings, especially early on in the, you know, 20 week range when most ultrasounds are done, they may be very subtle, and there may be nothing that, that tips the community obstetrician off that there's anything wrong.↗
clinicalThe specimen consisted of a 3.5 centimeter rib segment and a 5×3×5 centimeter lesion.↗
▶Ep 2 · 4:05
clinicalThe operation was complicated by a small postoperative pneumothorax which was managed with oxygen therapy.↗
▶Ep 2 · 4:18
clinicalThe patient was discharged on the 3rd postoperative day.↗
▶Ep 2 · 4:18
clinicalFinal pathology showed an atypical osteochondroma with normal underlying rib and negative margins.↗
▶Ep 2 · 4:25
clinicalAt 8 months follow-up, the patient was pain-free and back to normal activities.↗
▶Ep 2 · 4:25
quoteWe have successfully demonstrated how thorascopic assistance during chest wall excision is a useful technique for benign and malignant lesions to optimize cosmesis and margins.↗
▶Ep 2 · 5:36
quoteWe weren't sure what the diagnosis was. We, I mean, that's what we thought it probably was, but I just wasn't completely happy that that's what it was. It was so big and, and, and it was symptomatic in this case. So, uh, that's why we took the piece of rib.↗
Jack's statements about CICU / Post-op CHD Care84 statements
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 2 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 2 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 2 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 2 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 2 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 2 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 2 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 2 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 2 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 2 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 2 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 2 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 2 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 2 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 2 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 2 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 2 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 2 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 2 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 2 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 2 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 2 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 2 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 2 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 2 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 2 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 2 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 2 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 2 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 2 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 2 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 2 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 2 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 2 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 2 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 2 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 2 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 2 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 2 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 2 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 2 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 2 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 2 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 2 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 2 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 2 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 2 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 2 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 2 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 2 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 2 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 2 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 2 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 2 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 2 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 2 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 2 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 2 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 2 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 2 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 2 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 2 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 2 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 2 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 2 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 2 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 2 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 2 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 2 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 2 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 2 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 2 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 2 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 2 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 2 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 2 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 2 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 2 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 2 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 2 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 2 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 2 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 2 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
clinicalAt 20 weeks gestation when most ultrasounds are done, cloacal findings may be very subtle with nothing that tips the community obstetrician off that there's anything wrong↗
▶Ep 3 · 35:16
quoteI think you, you have to have a, a high index of suspicion to start looking for these subtle findings and, and I just think most people out there in the, in the real world aren't even thinking about this possibility.↗
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
▶Ep 14 · 30:18
epidemiologicalAt Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.↗
▶Ep 14 · 30:26
quoteOn routine ultrasound, these findings, especially early on in the, you know, 20 week range when most ultrasounds are done, they may be very subtle, and there may be nothing that, that tips the community obstetrician off that there's anything wrong.↗
▶Ep 14 · 30:26
opinionFindings of cloaca on routine 20-week ultrasound may be very subtle; community obstetricians may not suspect the diagnosis unless they have a high index of suspicion.↗
Jack's statements about Colorectal / ARM & Hirschsprung10 statements
clinicalAt 20 weeks gestation when most ultrasounds are done, cloacal findings may be very subtle with nothing that tips the community obstetrician off that there's anything wrong↗
▶Ep 17 · 35:00
clinicalAt 20 weeks gestation when most ultrasounds are done, cloacal findings may be very subtle with nothing that tips the community obstetrician off that there's anything wrong↗
▶Ep 17 · 35:16
quoteI think you, you have to have a, a high index of suspicion to start looking for these subtle findings and, and I just think most people out there in the, in the real world aren't even thinking about this possibility.↗
▶Ep 17 · 35:16
quoteI think you, you have to have a, a high index of suspicion to start looking for these subtle findings and, and I just think most people out there in the, in the real world aren't even thinking about this possibility.↗
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
▶Ep 38 · 30:18
epidemiologicalAt Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.↗
▶Ep 38 · 30:18
epidemiologicalAt Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.↗
▶Ep 38 · 30:26
opinionFindings of cloaca on routine 20-week ultrasound may be very subtle; community obstetricians may not suspect the diagnosis unless they have a high index of suspicion.↗
▶Ep 38 · 30:26
opinionFindings of cloaca on routine 20-week ultrasound may be very subtle; community obstetricians may not suspect the diagnosis unless they have a high index of suspicion.↗
▶Ep 38 · 30:26
quoteOn routine ultrasound, these findings, especially early on in the, you know, 20 week range when most ultrasounds are done, they may be very subtle, and there may be nothing that, that tips the community obstetrician off that there's anything wrong.↗
▶Ep 38 · 30:26
quoteOn routine ultrasound, these findings, especially early on in the, you know, 20 week range when most ultrasounds are done, they may be very subtle, and there may be nothing that, that tips the community obstetrician off that there's anything wrong.↗
Jack's statements about Congenital Lung Lesions (CPAM)14 statements
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 25 · 26:32
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 25 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 25 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 25 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 25 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 25 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 25 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 25 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 25 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 25 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 25 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 25 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
▶Ep 25 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
Jack's statements about Congenital Pulmonary Airway Malformation7 statements
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 21 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 21 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 21 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 21 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 21 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 21 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
Jack's statements about Congenital Pulmonary Airway Malformation7 statements
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 21 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 21 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 21 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 21 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 21 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 21 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
Jack's statements about CPAM (congenital Pulmonary Airway Malformation)7 statements
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 4 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 4 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 4 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 4 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 4 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 4 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
Jack's statements about Etiologies (Gastroschisis/NEC/Atresia/Volvulus)244 statements
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 10 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 10 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 10 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 10 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 10 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 10 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 10 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 10 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 10 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 10 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 10 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 10 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 10 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 10 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 10 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 10 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 10 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 10 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 10 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 10 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 10 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 10 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 10 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 10 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 10 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 10 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 10 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 10 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 10 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 10 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 10 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 10 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 10 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 10 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 10 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 10 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 10 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 10 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 10 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 10 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 10 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 10 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 10 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 10 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 10 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 10 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 10 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 10 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 10 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 10 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 10 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 10 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 10 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 10 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 10 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 10 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 10 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 10 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 10 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 10 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 10 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 10 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 10 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 10 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 10 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 10 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 10 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 10 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 10 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 10 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 10 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 10 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 10 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 30 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 30 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 30 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 30 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 30 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 30 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 30 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 30 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 30 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 30 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 30 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 30 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 30 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 30 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 30 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 30 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 30 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 30 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 30 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 30 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 30 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 30 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 30 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 30 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 30 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 30 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 30 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 30 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 30 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 30 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 30 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 30 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 30 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 30 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 30 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 30 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 30 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 30 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 30 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 30 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 30 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 30 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 30 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 30 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 30 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 30 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 30 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 30 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 30 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 30 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 30 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 30 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 30 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 30 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 30 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 30 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 30 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 30 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 30 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 30 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 30 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 30 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 30 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 30 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 30 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 30 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 30 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 30 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 30 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 30 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 30 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 30 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 30 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 30 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 30 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 30 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 30 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 30 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 30 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 30 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 30 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 30 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 30 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 30 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 30 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 30 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 30 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 30 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 30 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 30 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 30 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 30 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 30 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 30 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 30 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 30 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 30 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 30 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 30 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 30 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 30 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 30 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 30 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 30 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 30 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 30 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 30 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 30 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 30 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 30 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 30 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 30 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 30 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 30 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 30 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 30 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 30 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 30 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 30 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 30 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 30 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 30 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 30 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 30 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 30 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 30 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 30 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 30 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 30 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 30 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 30 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 30 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 30 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 30 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 30 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 30 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 30 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 30 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 30 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 30 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 30 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 30 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 30 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 30 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 30 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 30 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 30 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 30 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 30 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 30 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 30 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 30 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 30 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 30 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 30 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 30 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 30 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 30 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 30 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 30 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 30 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 30 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 30 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 30 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 30 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 30 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 30 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
▶Ep 30 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
quoteI don't think you can underestimate the fact that you're taking an asymptomatic child and subjecting them to an operation that is associated with potential risk.↗
▶Ep 17 · 26:49
quoteI think you have to sit down with the family and say there isn't a clear right answer on this one.↗
▶Ep 17 · 28:25
clinicalFeatures suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.↗
▶Ep 17 · 28:59
clinicalFeatures suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.↗
▶Ep 17 · 30:56
clinicalDr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.↗
▶Ep 17 · 31:51
clinicalIf a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.↗
▶Ep 17 · 32:49
quoteDoes that mean that every single child with an asymptomatic CPAM should be subjected to a lobectomy so that you make your operation easier for yourself? I don't think so.↗
Jack's statements about Gastroschisis160 statements
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 4 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 4 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 4 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 4 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 4 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 4 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 4 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 4 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 4 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 4 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 4 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 4 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 4 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 4 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 4 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 4 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 4 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 4 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 4 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 4 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 4 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 4 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 4 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 4 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 4 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 4 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 4 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 4 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 4 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 4 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 4 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 4 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 4 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 4 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 4 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 4 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 4 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 4 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 4 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 4 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 4 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 4 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 4 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 4 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 4 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 4 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 4 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 4 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 4 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 4 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 4 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 4 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 4 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 4 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 4 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 4 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 4 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 4 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 4 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 4 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 4 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 4 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 4 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 4 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 4 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 4 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 4 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 4 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 4 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 4 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 4 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 4 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 4 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 13 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 13 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 13 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 13 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 13 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 13 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 13 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 13 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 13 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 13 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 13 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 13 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 13 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 13 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 13 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 13 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 13 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 13 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 13 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 13 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 13 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 13 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 13 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 13 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 13 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 13 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 13 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 13 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 13 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 13 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 13 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 13 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 13 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 13 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 13 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 13 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 13 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 13 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 13 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 13 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 13 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 13 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 13 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 13 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 13 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 13 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 13 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 13 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 13 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 13 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 13 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 13 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 13 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 13 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 13 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 13 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 13 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 13 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 13 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 13 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 13 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 13 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 13 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 13 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 13 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 13 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 13 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 13 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 13 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 13 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 13 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 13 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 13 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 13 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 13 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 13 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 13 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 13 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 13 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 13 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 13 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 13 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 13 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 13 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
Jack's statements about Gastroschisis160 statements
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 4 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 4 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 4 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 4 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 4 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 4 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 4 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 4 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 4 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 4 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 4 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 4 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 4 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 4 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 4 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 4 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 4 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 4 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 4 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 4 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 4 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 4 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 4 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 4 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 4 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 4 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 4 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 4 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 4 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 4 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 4 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 4 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 4 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 4 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 4 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 4 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 4 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 4 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 4 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 4 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 4 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 4 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 4 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 4 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 4 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 4 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 4 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 4 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 4 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 4 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 4 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 4 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 4 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 4 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 4 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 4 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 4 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 4 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 4 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 4 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 4 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 4 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 4 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 4 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 4 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 4 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 4 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 4 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 4 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 4 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 4 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 4 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 4 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 12 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 12 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 12 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 12 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 12 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 12 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 12 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 12 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 12 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 12 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 12 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 12 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 12 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 12 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 12 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 12 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 12 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 12 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 12 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 12 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 12 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 12 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 12 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 12 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 12 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 12 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 12 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 12 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 12 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 12 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 12 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 12 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 12 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 12 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 12 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 12 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 12 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 12 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 12 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 12 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 12 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 12 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 12 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 12 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 12 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 12 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 12 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 12 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 12 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 12 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 12 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 12 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 12 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 12 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 12 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 12 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 12 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 12 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 12 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 12 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 12 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 12 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 12 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 12 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 12 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 12 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 12 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 12 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 12 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 12 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 12 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 12 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 12 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 12 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 12 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 12 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 12 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 12 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 12 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 12 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 12 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 12 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 12 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 12 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
Jack's statements about Intestinal Rehab244 statements
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 17 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 17 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 17 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 17 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 17 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 17 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 17 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 17 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 17 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 17 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 17 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 17 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 17 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 17 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 17 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 17 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 17 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 17 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 17 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 17 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 17 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 17 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 17 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 17 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 17 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 17 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 17 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 17 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 17 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 17 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 17 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 17 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 17 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 17 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 17 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 17 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 17 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 17 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 17 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 17 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 17 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 17 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 17 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 17 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 17 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 17 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 17 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 17 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 17 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 17 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 17 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 17 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 17 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 17 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 17 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 17 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 17 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 17 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 17 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 17 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 17 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 17 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 17 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 17 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 17 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 17 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 17 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 17 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 17 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 17 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 17 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 17 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 17 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 39 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 39 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 39 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 39 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 39 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 39 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 39 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 39 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 39 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 39 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 39 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 39 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 39 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 39 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 39 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 39 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 39 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 39 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 39 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 39 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 39 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 39 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 39 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 39 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 39 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 39 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 39 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 39 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 39 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 39 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 39 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 39 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 39 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 39 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 39 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 39 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 39 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 39 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 39 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 39 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 39 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 39 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 39 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 39 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 39 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 39 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 39 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 39 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 39 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 39 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 39 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 39 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 39 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 39 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 39 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 39 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 39 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 39 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 39 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 39 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 39 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 39 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 39 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 39 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 39 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 39 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 39 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 39 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 39 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 39 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 39 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 39 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 39 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 39 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 39 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 39 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 39 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 39 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 39 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 39 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 39 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 39 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 39 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 39 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 39 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 39 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 39 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 39 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 39 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 39 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 39 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 39 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 39 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 39 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 39 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 39 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 39 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 39 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 39 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 39 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 39 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 39 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 39 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 39 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 39 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 39 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 39 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 39 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 39 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 39 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 39 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 39 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 39 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 39 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 39 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 39 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 39 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 39 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 39 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 39 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 39 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 39 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 39 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 39 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 39 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 39 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 39 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 39 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 39 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 39 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 39 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 39 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
▶Ep 39 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 39 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni↗
▶Ep 39 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 39 · 45:08
clinicalPentalogy of Cantrell omphaloceles tend to be more superiorly placed↗
▶Ep 39 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 39 · 45:19
clinicalMost pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele↗
▶Ep 39 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 39 · 45:31
clinicalIn pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later↗
▶Ep 39 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 39 · 46:08
clinicalReflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia↗
▶Ep 39 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 39 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect↗
▶Ep 39 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 39 · 46:55
clinicalAfter G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux↗
▶Ep 39 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 39 · 47:15
clinicalFundoplication in a child with unrepaired giant omphalocele is extremely difficult because the liver is midline and access to the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 39 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 39 · 47:42
clinicalGJ tube is a better short-term solution than fundoplication for reflux in unrepaired omphalocele, with fundoplication performed at the time of definitive abdominal wall repair↗
▶Ep 39 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 39 · 48:08
clinicalIn giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 39 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 39 · 48:49
clinicalNon-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary↗
▶Ep 39 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 39 · 49:06
opinionInversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures↗
▶Ep 39 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 39 · 49:18
clinicalChildren with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 39 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 39 · 49:44
clinicalIf a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved↗
▶Ep 39 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 39 · 50:16
clinicalDuring omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 39 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 39 · 50:38
clinicalKinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20↗
▶Ep 39 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 39 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 39 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
▶Ep 39 · 51:09
clinicalFor partial omphalocele reduction with liver still protruding at the top, a temporary Gore-Tex or silastic patch can be sewn on with skin closed over it, then removed in 1-2 weeks for definitive fascial closure↗
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 3 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 3 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 3 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 3 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 3 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 3 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 3 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 3 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 3 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 3 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 3 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 3 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 3 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 3 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 3 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 3 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 3 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 3 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 3 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 3 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 3 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 3 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 3 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 3 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 3 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 3 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 3 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 3 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 3 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 3 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 3 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 3 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 3 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 3 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 3 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 3 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 3 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 3 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 3 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 3 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 3 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 3 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 3 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 3 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 3 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 3 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 3 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 3 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 3 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 3 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 3 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 3 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 3 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 3 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 3 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 3 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 3 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 3 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 3 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 3 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 3 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 3 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 3 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 3 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 3 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 3 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 3 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 3 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 3 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 3 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 3 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 3 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 3 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 8 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 8 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 8 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 8 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 8 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 8 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 8 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 8 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 8 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 8 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 8 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 8 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 8 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 8 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 8 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 8 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 8 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 8 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 8 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 8 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 8 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 8 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 8 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 8 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 8 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 8 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 8 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 8 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 8 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 8 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 8 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 8 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 8 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 8 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 8 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 8 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 8 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 8 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 8 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 8 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 8 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 8 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 8 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 8 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 8 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 8 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 8 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 8 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 8 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 8 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 8 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 8 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 8 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 8 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 8 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 8 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 8 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 8 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 8 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 8 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 8 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 8 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 8 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 8 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 8 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 8 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 3 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis↗
▶Ep 3 · 5:04
quoteWill the diagnosis change the location of delivery? Will the diagnosis change the timing of delivery? Will the diagnosis change the mode of delivery? In other words, do you need a cesarean section? And is there a role for some kind of fetal intervention?↗
▶Ep 3 · 5:46
clinicalEarly papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 3 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section↗
▶Ep 3 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis↗
▶Ep 3 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 3 · 7:07
clinicalThe mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 3 · 7:30
clinicalLabor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies↗
▶Ep 3 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 3 · 10:01
clinicalDuring transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 3 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 3 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 3 · 11:47
clinicalCurrent technique uses pre-formed silos with fentanyl or morphine sedation without intubation in awake babies, slowly pushing bowel in while monitoring intraabdominal pressure to keep it below 20↗
▶Ep 3 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 3 · 13:40
clinicalAnthony Sandler championed the sutureless plastic closure approach after training in Toronto↗
▶Ep 3 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 3 · 17:25
clinicalLeaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure↗
▶Ep 3 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 3 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia with dilated but not thick-walled bowel, and late atresia from a constricting defect causing ischemia and potentially vanishing gastroschisis↗
▶Ep 3 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years due to intestinal failure centers, better TPN that doesn't damage the liver, and improved sepsis control↗
▶Ep 3 · 20:54
clinicalThree management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months↗
▶Ep 3 · 21:15
opinionThere is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized↗
▶Ep 3 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 3 · 21:35
clinicalIf bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair↗
▶Ep 3 · 22:01
clinicalStomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 3 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location↗
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 3 · 24:55
clinicalMetoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility↗
▶Ep 3 · 25:02
clinicalA UK study showed cisapride shortened time to bowel function, but cisapride is no longer available↗
▶Ep 3 · 25:37
clinicalToronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 3 · 25:57
clinicalAt 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI↗
▶Ep 3 · 26:44
clinicalIf still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions↗
▶Ep 3 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 3 · 28:03
clinicalUndescended testis (usually right) in gastroschisis is directed down into the pelvis during reduction; in about half the cases it finds its way to the scrotum↗
▶Ep 3 · 28:18
quoteOne of the most amazing things in the human body.↗
▶Ep 3 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 3 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients↗
▶Ep 3 · 29:47
clinicalSmall omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 3 · 30:05
clinicalThere is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles↗
▶Ep 3 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 3 · 31:04
clinicalFor giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based↗
▶Ep 3 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists↗
▶Ep 3 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support↗
▶Ep 3 · 33:28
clinicalStuart Lacy established intraabdominal pressure guideline of 20 mmHg based on rabbit studies in the 1980s, then validated in prospective study in children showing improved outcomes↗
▶Ep 3 · 33:56
clinicalLacy also described increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 3 · 34:02
clinicalIntraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number↗
▶Ep 3 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 3 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure↗
▶Ep 3 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side↗
▶Ep 3 · 37:43
clinicalDuoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation↗
▶Ep 3 · 38:15
clinicalIndications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce↗
▶Ep 3 · 38:49
clinicalMushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily↗
▶Ep 3 · 39:26
clinicalSigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up↗
▶Ep 3 · 40:19
clinicalTiming of delayed omphalocele repair depends on patient specifics; some can be repaired at 6-8 months if defect reduces spontaneously, others require waiting 3-4 years for cardiac or pulmonary optimization↗
▶Ep 3 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 3 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged as a first step by incising the lower edge fascia, closing skin, and allowing more spontaneous reduction before definitive repair↗
▶Ep 3 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and worsening the situation↗
▶Ep 3 · 43:35
clinicalIn omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly↗
▶Ep 3 · 44:01
clinicalSurgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh↗
▶Ep 3 · 44:19
clinicalStratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis↗
▶Ep 3 · 44:44
clinicalOmphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed↗
▶Ep 3 · 45:24
clinicalFor pentalogy of Cantrell patients, escharotic therapy is used due to cardiac problems; cardiac surgeons can patch the diaphragm from above through sternotomy, then abdominal wall is repaired later after cardiac optimization↗
▶Ep 3 · 46:08
clinicalReflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally↗
▶Ep 3 · 46:30
clinicalInterventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect, then convert to GJ tube after maturation↗
▶Ep 3 · 47:15
clinicalFundoplication in a child with a large omphalocele defect is extremely difficult because the liver is midline and accessing the hiatus is nearly impossible, especially with cardiac disease and congested liver↗
▶Ep 3 · 47:42
clinicalGJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization↗
▶Ep 3 · 48:08
clinicalIn large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux↗
▶Ep 3 · 48:49
clinicalNon-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation↗
▶Ep 3 · 49:06
clinicalInversion appendectomy during Ladd procedure makes sense if the appendix is encountered, as several omphalocele patients have developed perforated appendicitis with delayed diagnosis due to abnormal appendix location↗
▶Ep 3 · 49:44
clinicalIf a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved↗
▶Ep 3 · 50:10
clinicalHepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful↗
▶Ep 3 · 50:31
clinicalKinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20↗
▶Ep 3 · 50:56
clinicalIn immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement↗
▶Ep 3 · 51:09
clinicalFor partial reductions with liver still protruding superiorly, Gore-Tex or silastic can be sewn on, skin closed over it, then removed in 1-2 weeks after stretching allows fascial closure↗
Abdominal Wall Defects with Dr. Jacob Langer
▶Ep 9 · 3:46
quoteDiversification is not only important in your financial portfolio, it's also important in the rest of your life.↗
▶Ep 9 · 4:36
clinicalWith gastroschisis, the main issue is that the bowel gets damaged through fetal life↗
▶Ep 9 · 4:46
clinicalMost gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes↗
▶Ep 9 · 5:46
clinicalEarly papers showed a benefit to cesarean section in gastroschisis, but cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit↗
▶Ep 9 · 6:13
clinicalMany studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis↗
▶Ep 9 · 6:29
clinicalThere has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis↗
▶Ep 9 · 6:58
clinicalToronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor↗
▶Ep 9 · 7:07
clinicalThe mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel↗
▶Ep 9 · 7:30
clinicalIn gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies↗
▶Ep 9 · 8:36
clinicalMost evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis↗
▶Ep 9 · 10:01
clinicalDuring transport of gastroschisis patients, it's important for the baby to be nursed on his or her side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel↗
▶Ep 9 · 10:57
clinicalFor gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel↗
▶Ep 9 · 11:21
clinicalAdrian Bianchi first described bedside closure for gastroschisis↗
▶Ep 9 · 11:29
clinicalUsing forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby↗
▶Ep 9 · 11:47
clinicalThe current approach uses pre-formed silos, slowly pushing on them with fentanyl or morphine sedation in an awake baby, aiming to keep intraabdominal pressure below 20↗
▶Ep 9 · 12:27
clinicalIf reduction is successful with good pressure and perfusion, the silo can be removed immediately and the umbilical cord stump used to cover the hole with a Duoderm dressing left for about 5 days↗
▶Ep 9 · 14:56
clinicalUmbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia↗
▶Ep 9 · 16:12
clinicalSome gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later↗
▶Ep 9 · 17:25
clinicalThe disadvantage of leaving a silo on for 24-48 hours is that the abdominal wall defect gets stretched out and bigger, taking longer to close with plastic closure↗
▶Ep 9 · 18:51
epidemiologicalThe incidence of intestinal atresia in gastroschisis is between 5 and 10%↗
▶Ep 9 · 19:01
clinicalThere are two types of intestinal atresia in gastroschisis: early-onset atresia where bowel gets very dilated but not thick-walled, and late atresia where the abdominal wall defect becomes very small causing ischemia and potentially vanishing gastroschisis↗
▶Ep 9 · 20:15
clinicalThe prognosis for short bowel syndrome has improved dramatically over the last 10-15 years because of intestinal failure centers, better TPN that doesn't damage the liver as much, and control of sepsis↗
▶Ep 9 · 20:54
clinicalFor atresia in gastroschisis, there are three management choices: repair at time of closure, bring out stomas, or drop everything back in and repair the atresia later↗
▶Ep 9 · 21:15
opinionThere's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence↗
▶Ep 9 · 21:21
quoteUsually when you say individualize, it means nobody knows what the right answer is.↗
▶Ep 9 · 21:35
clinicalIf bowel looks good without much peel, primary repair of atresia at initial closure is appropriate; if there's concern, drop it back in and repair later↗
▶Ep 9 · 22:01
clinicalStomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose↗
▶Ep 9 · 22:50
clinicalThe umbilicus is the preferred site for neonatal stomas because it results in a scar that would have been there anyway and is convenient for appliance placement↗
▶Ep 9 · 23:00
clinicalNeonatal stomas prolapse no matter where they are placed↗
▶Ep 9 · 24:27
clinicalThree weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks↗
▶Ep 9 · 25:02
clinicalA UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available↗
▶Ep 9 · 25:02
clinicalMetoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain↗
▶Ep 9 · 25:37
clinicalA randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis↗
▶Ep 9 · 25:57
clinicalAt 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive↗
▶Ep 9 · 26:44
clinicalIf still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down↗
▶Ep 9 · 27:21
opinionGoing in too early on gastroschisis patients with prolonged ileus is a mistake↗
▶Ep 9 · 28:03
clinicalIn gastroschisis, it's usually the right testis that is extruded, and in about half the cases it finds its way down into the scrotum after being reduced↗
▶Ep 9 · 28:43
clinicalOmphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis↗
▶Ep 9 · 28:59
clinicalKaryotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients↗
▶Ep 9 · 29:47
clinicalSmall omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles↗
▶Ep 9 · 30:05
clinicalFor small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery↗
▶Ep 9 · 30:23
clinicalSmall omphaloceles are simple to repair surgically↗
▶Ep 9 · 31:04
clinicalFor giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based↗
▶Ep 9 · 31:42
clinicalGiant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists↗
▶Ep 9 · 31:55
clinicalPulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally↗
▶Ep 9 · 32:09
clinicalSome giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support↗
▶Ep 9 · 32:38
clinicalThe goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure↗
▶Ep 9 · 33:14
clinicalIntraabdominal pressure monitoring is very helpful in omphalocele management↗
▶Ep 9 · 33:28
clinicalStuart Lacy established the guideline of keeping intraabdominal pressure below 20 mmHg based on rabbit studies in the 1980s, which showed improved outcomes in a prospective study in children↗
▶Ep 9 · 33:56
clinicalLacy also described an increase in central venous pressure of more than 4 as a concerning threshold↗
▶Ep 9 · 34:02
clinicalIntraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure↗
▶Ep 9 · 34:17
clinicalThe trend of pressure is more important than the absolute number during reduction↗
▶Ep 9 · 34:43
clinicalPrimary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia↗
▶Ep 9 · 35:07
clinicalThe Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching↗
▶Ep 9 · 35:44
clinicalSequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side↗
▶Ep 9 · 37:34
clinicalUsing Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation↗
▶Ep 9 · 38:12
clinicalIndications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible↗
▶Ep 9 · 38:49
clinicalMushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible↗
▶Ep 9 · 39:26
clinicalSigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles↗
▶Ep 9 · 39:45
clinicalWith escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia↗
▶Ep 9 · 40:19
clinicalTiming of definitive omphalocele repair after escharotic therapy depends on the individual child, ranging from 6-8 months to 3-4 years depending on defect size and medical comorbidities↗
▶Ep 9 · 41:10
clinicalMushroom-shaped omphaloceles never reduce spontaneously and stay large↗
▶Ep 9 · 41:20
clinicalFor mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair↗
▶Ep 9 · 43:06
clinicalFormal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur↗
▶Ep 9 · 43:35
clinicalIn omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement↗
▶Ep 9 · 44:01
clinicalSurgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene↗
