Jack Langer
1500 statements · 14 topics
Colorectal / ARM & Hirschsprung
· guest expert
Congenital Lung Lesions (CPAM)
· guest expert
Fetal Surgery
· guest expert
Featured statements
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
clinical ·
Abdominal Wall Defects
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
clinical ·
Abdominal Wall Defects
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
clinical ·
CICU / Post-op CHD Care
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
Inversion 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
clinical ·
Gastroschisis
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Jack's statements about Abdominal Wall Defects 320 statements
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Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
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Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
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Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
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Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
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Will 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?
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Quotation
Will 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?
Open
Statement
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
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
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Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
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There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
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There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
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Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
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Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
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The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
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The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
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Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
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Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
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Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
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Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
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During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
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During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
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Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
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Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
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Current 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
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Current 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
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If 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
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If 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
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Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
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Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
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Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
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Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
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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
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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
Open
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The 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
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The 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
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Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
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Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
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There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
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There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
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If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
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If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
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Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
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Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
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The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
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The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
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Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
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Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
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Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
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Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
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A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
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A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
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Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
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Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
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At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
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At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
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If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
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If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
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Undescended 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
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Undescended 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
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Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
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Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
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Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
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Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
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Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
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Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
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There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
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There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
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For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
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For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
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Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
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Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
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Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
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Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
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Stuart 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
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Stuart 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
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Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
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Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
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Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
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Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
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Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
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Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
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The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
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The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
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Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
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Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
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Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
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Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
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Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
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Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
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Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
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Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
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Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
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Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
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Timing 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
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Timing 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
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For 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
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For 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
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Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
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Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
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In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
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In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
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Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
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Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
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Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
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Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
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Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
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Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
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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
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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
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Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
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Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
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Interventional 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
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Interventional 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
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Fundoplication 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
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Fundoplication 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
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GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
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GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
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In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
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In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
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Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
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Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
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Inversion 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
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Inversion 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
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If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
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If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
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Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
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Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
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Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
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Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
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In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
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In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
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For 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
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For 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
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Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
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Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
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Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
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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
Open
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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
Open
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Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
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Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
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There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
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There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
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Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
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Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
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The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
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The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
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In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
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In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
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Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
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Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
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During 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
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During 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
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For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
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For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
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Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
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Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
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The 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
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The 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
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If 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
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If 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
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Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
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Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
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Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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The 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
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The 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
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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
Open
Statement
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
Open
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The 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
Open
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The 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
Open
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For 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
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For 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
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There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
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There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
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If 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
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If 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
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Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
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Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
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The 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
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The 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
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Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
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Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
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A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
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Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
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A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
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Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
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A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
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A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
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At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
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At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
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If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
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If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
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In 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
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In 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
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Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
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Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
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Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
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Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
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Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Statement
For 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
Open
Jack's statements about Anorectal Malformation 5 statements
Open the Anorectal Malformation collection →
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
At 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
Open
Quotation
I 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.
Open
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
Statement
At Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.
Open
Quotation
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.
Open
Statement
Findings 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.
Open
Jack's statements about Chest Pain 18 statements
Open the Chest Pain collection →
Tricks - Thoracoscopic-Assisted Partial Rib Resection - Jack Langer
Statement
Chest X-ray showed an ill-defined 3×5×5 centimeter calcified lesion in the left lower lung zone.
Open
Statement
CT scan showed the lesion was pedunculated and exophytic, arising from the internal aspect of the left 6th rib.
Open
Statement
A 22 gauge needle was passed through the chest wall to map out the smallest incision possible and better define the margins.
Open
Statement
The intercostal muscles and neurovascular bundles were separated from above and below the 6th rib.
Open
Statement
The rib was cut anterior and posterior to the stock of the lesion, and the rib segment and lesion were extracted.
Open
Statement
The specimen consisted of a 3.5 centimeter rib segment and a 5×3×5 centimeter lesion.
Open
Statement
The operation was complicated by a small postoperative pneumothorax which was managed with oxygen therapy.
Open
Statement
Final pathology showed an atypical osteochondroma with normal underlying rib and negative margins.
Open
Quotation
We have successfully demonstrated how thorascopic assistance during chest wall excision is a useful technique for benign and malignant lesions to optimize cosmesis and margins.
Open
Quotation
We 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.
Open
Jack's statements about CICU / Post-op CHD Care 84 statements
Open the CICU / Post-op CHD Care collection →
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
The 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
Open
Statement
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
Open
Statement
The 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
Open
Statement
For 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
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
If 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
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The 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
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
In 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Jack's statements about Cloaca 5 statements
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
At 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
Open
Quotation
I 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.
Open
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
Statement
At Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.
Open
Statement
Findings 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.
Open
Quotation
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.
Open
Jack's statements about Colorectal / ARM & Hirschsprung 8 statements
Open the Colorectal / ARM & Hirschsprung collection →
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
At 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
Open
Quotation
I 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.
Open
Prenatal Imaging and Counseling: Cloaca and Complex ARMs 2015
Statement
At Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.
Open
Statement
At Sick Kids (Toronto), an increasing number of cloaca cases have prenatal diagnosis, but the majority still do not.
Open
Quotation
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.
Open
Statement
Findings 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.
Open
Quotation
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.
Open
Statement
Findings 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.
Open
Jack's statements about Congenital Lung Lesions (CPAM) 14 statements
Open the Congenital Lung Lesions (CPAM) collection →
The Full Story on CPAMs
Quotation
I 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.
Open
Quotation
I 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.
Open
Quotation
I think you have to sit down with the family and say there isn't a clear right answer on this one.
Open
Quotation
I think you have to sit down with the family and say there isn't a clear right answer on this one.
Open
Statement
Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.
Open
Statement
Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.
Open
Statement
Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.
Open
Statement
Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.
Open
Statement
Dr. 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.
Open
Statement
Dr. 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.
Open
Statement
If 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.
Open
Statement
If 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.
Open
Quotation
Does 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.
Open
Quotation
Does 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.
Open
Jack's statements about Congenital Pulmonary Airway Malformation 7 statements
Open the Congenital Pulmonary Airway Malformation collection →
The Full Story on CPAMs
Quotation
I 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.
Open
Quotation
I think you have to sit down with the family and say there isn't a clear right answer on this one.
Open
Statement
Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.
Open
Statement
Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.
Open
Statement
Dr. 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.
Open
Statement
If 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.
Open
Quotation
Does 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.
Open
Jack's statements about Etiologies (Gastroschisis/NEC/Atresia/Volvulus) 244 statements
Open the Etiologies (Gastroschisis/NEC/Atresia/Volvulus) collection →
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Quotation
Will 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?
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Current 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
Open
Statement
If 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
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
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
Open
Statement
The 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
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
Undescended 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Stuart 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
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Timing 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
Open
Statement
For 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
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
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
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Interventional 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
Open
Statement
Fundoplication 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
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Inversion 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
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
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
Open
Statement
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
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
For 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
Open
Statement
For 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
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
If 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
Open
Statement
If 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
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
In 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
Open
Statement
In 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Statement
For 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
Open
Jack's statements about Fetal Surgery 7 statements
Open the Fetal Surgery collection →
The Full Story on CPAMs
Quotation
I 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.
Open
Quotation
I think you have to sit down with the family and say there isn't a clear right answer on this one.
Open
Statement
Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.
Open
Statement
Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.
Open
Statement
Dr. 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.
Open
Statement
If 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.
Open
Quotation
Does 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.
Open
Jack's statements about Gastroschisis 160 statements
Open the Gastroschisis collection →
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Quotation
Will 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?
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Current 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
Open
Statement
If 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
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
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
Open
Statement
The 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
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
Undescended 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Stuart 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
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Timing 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
Open
Statement
For 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
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
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
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Interventional 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
Open
Statement
Fundoplication 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
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Inversion 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
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
The 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
Open
Statement
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
Open
Statement
The 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
Open
Statement
For 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
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
If 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
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The 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
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
In 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Jack's statements about Intestinal Rehab 320 statements
Open the Intestinal Rehab collection →
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Quotation
Will 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?
Open
Quotation
Will 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?
Open
Statement
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
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Current 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
Open
Statement
Current 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
Open
Statement
If 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
Open
Statement
If 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
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
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
Open
Statement
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
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
Undescended 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
Open
Statement
Undescended 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Stuart 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
Open
Statement
Stuart 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
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Timing 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
Open
Statement
Timing 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
Open
Statement
For 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
Open
Statement
For 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
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
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
Open
Statement
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
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Interventional 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
Open
Statement
Interventional 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
Open
Statement
Fundoplication 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
Open
Statement
Fundoplication 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
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Inversion 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
Open
Statement
Inversion 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
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Statement
For 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
Open
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
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
Open
Statement
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
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
For 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
Open
Statement
For 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
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
If 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
Open
Statement
If 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
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The 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
Open
Statement
The 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
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
In 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
Open
Statement
In 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Statement
For 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
Open
Jack's statements about Omphalocele 160 statements
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Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Quotation
Will 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?
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Current 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
Open
Statement
If 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
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
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
Open
Statement
The 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
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
Undescended 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Stuart 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
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Timing 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
Open
Statement
For 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
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
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
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Interventional 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
Open
Statement
Fundoplication 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
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Inversion 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
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
The 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
Open
Statement
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
Open
Statement
The 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
Open
Statement
For 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
Open
Statement
There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
Open
Statement
If 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
Open
Statement
Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The 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
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
Open
Statement
A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
Open
Statement
A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
Open
Statement
If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
Open
Statement
In 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
Open
Statement
Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
Open
Statement
For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
Open
Statement
Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
Open
Statement
The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
Open
Statement
Stuart 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
Open
Statement
Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
Open
Statement
Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
Open
Statement
Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
Open
Statement
Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
Open
Statement
Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
Open
Statement
With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
Open
Statement
Timing 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
Open
Statement
For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
Open
Statement
In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
Open
Statement
Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
Open
Statement
Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
Open
Statement
In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
Open
Statement
Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
Open
Statement
Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
Open
Statement
After G-tube maturation, a GJ tube can be placed to allow feeding despite severe reflux
Open
Statement
Fundoplication 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
Open
Statement
GJ 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
Open
Statement
In giant omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not associated with risk of midgut volvulus, so Ladd's procedure is not necessary
Open
Statement
Inversion appendectomy during omphalocele repair makes sense if the surgeon performs appendectomies during Ladd's procedures
Open
Statement
Children with repaired omphaloceles can develop perforated appendicitis with delayed diagnosis due to abnormal appendix location
Open
Statement
If a child with omphalocele has renal abnormalities requiring potential Mitrofanoff procedure, the appendix should be preserved
Open
Statement
During omphalocele reduction with liver, hepatic veins are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during omphalocele reduction is prevented by using intraabdominal pressure monitoring and not being too aggressive with reduction when pressures exceed 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Jack's statements about Single Ventricle / HLHS 148 statements
Open the Single Ventricle / HLHS collection →
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Open
Quotation
Will 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?
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During transport, gastroschisis babies should be nursed on their side, usually right side down, to prevent kinking of the mesentery and ischemia of the bowel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
Current 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
Open
Statement
If 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
Open
Statement
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Open
Statement
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
Open
Statement
Leaving a silo on for 24-48 hours causes the abdominal wall defect to stretch and get bigger, taking longer to close with plastic closure
Open
Statement
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
Open
Statement
The 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
Open
Statement
Three management options for atresia in gastroschisis: repair at time of closure, bring out stomas, or reduce everything and repair atresia in a couple months
Open
Statement
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
Open
Statement
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Open
Statement
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
Open
Statement
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Open
Statement
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Open
Statement
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
Open
Statement
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Open
Statement
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
Open
Statement
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
Open
Statement
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Open
Statement
Undescended 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
Open
Statement
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Open
Statement
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Open
Statement
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
Open
Statement
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Open
Statement
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Open
Statement
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Open
Statement
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
Open
Statement
Stuart 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
Open
Statement
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Open
Statement
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Open
Statement
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
Open
Statement
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Open
Statement
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Open
Statement
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Open
Statement
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Open
Statement
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Open
Statement
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
Open
Statement
Timing 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
Open
Statement
For 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
Open
Statement
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
Open
Statement
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Open
Statement
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Open
Statement
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Open
Statement
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed
Open
Statement
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
Open
Statement
Reflux is very common in omphalocele patients, especially those with bad hearts or pulmonary hypoplasia who don't eat normally
Open
Statement
Interventional 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
Open
Statement
Fundoplication 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
Open
Statement
GJ tube is a better short-term solution for reflux in omphalocele; fundoplication can be performed when fixing the abdominal wall defect after medical optimization
Open
Statement
In large omphaloceles with midline liver, the liver can put pressure on the duodenum or pylorus causing mechanical gastric outlet obstruction that worsens reflux
Open
Statement
Non-rotation in omphalocele is not a problem because it's not associated with risk of midgut volvulus, unlike malrotation
Open
Statement
Inversion 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
Open
Statement
If a child has renal abnormality requiring potential Mitrofanoff, the appendix should be preserved
Open
Statement
Hepatic veins in omphalocele are very superficial and can be injured during fascial dissection if not careful
Open
Statement
Kinking of hepatic veins during reduction has not been a problem when using intraabdominal pressure monitoring and not being too aggressive with pressures above 20
Open
Statement
In immediate omphalocele reduction, fascial edges can usually be approximated at the bottom but often not at the top, requiring patch placement
Open
Statement
For 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
Open
Abdominal Wall Defects with Dr. Jacob Langer
Quotation
Diversification is not only important in your financial portfolio, it's also important in the rest of your life.
Open
Statement
Most gastroschisis patients don't have any other associated anomalies, and it's pretty rare to have abnormal chromosomes
Open
Statement
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
Open
Statement
Many studies have failed to show an advantage to cesarean section, and most people nowadays would not do routine cesarean section for gastroschisis
Open
Statement
There has not been any large randomized trial looking specifically at the issue of early delivery in gastroschisis
Open
Statement
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
Open
Statement
The mean gestational age of onset of labor is a lot earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Open
Statement
In gastroschisis pregnancies, labor can usually be successfully induced at 37 weeks, unlike regular pregnancies
Open
Statement
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
Open
Statement
During 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
Open
Statement
For gastroschisis, bedside closure is the first choice if the bowel is not too thickened and there's not too much peel
Open
Statement
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
Open
Statement
The 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
Open
Statement
If 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
Open
Statement
Umbilical hernias from plastic closure generally close by age 2 or 3 years, just like any umbilical hernia
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Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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The 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
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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
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The 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
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For 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
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There's no good evidence for optimal management of atresia in gastroschisis because it's such a rare occurrence
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If 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
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Stomas are brought out only when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
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The 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
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Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
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A UK study showed cisapride helped gastroschisis patients achieve bowel function more quickly, but cisapride is no longer available
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Metoclopramide (Reglan) can be given intravenously for gastroschisis hypomotility, unlike oral prokinetics where absorption is uncertain
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A randomized prospective trial is currently underway to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
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At 4 weeks without bowel function, contrast enema is performed to look for mechanical obstruction, sometimes followed by upper GI if enema is inconclusive
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If still no bowel function at 6 weeks, laparotomy is typically performed, often finding adhesions which are taken down
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In 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
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Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
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Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely performed for omphalocele patients
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Small omphaloceles without liver are more likely to be associated with abnormal chromosomes than large omphaloceles
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For small omphaloceles, there's no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery
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For giant omphaloceles (defined as any omphalocele with a lot of liver out), most surgeons recommend cesarean section, though this is not evidence-based
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Giant omphaloceles should be delivered at a perinatal center because they need a pediatric surgeon and experienced neonatologists
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Pulmonary hypoplasia is associated with giant omphaloceles and is very difficult to diagnose prenatally
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Some giant omphalocele patients have severe pulmonary hypoplasia requiring early intubation and respiratory support
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The goal in omphalocele management is to reduce viscera without injury from direct trauma or increased intraabdominal pressure
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Stuart 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
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Lacy also described an increase in central venous pressure of more than 4 as a concerning threshold
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Intraabdominal pressure can be measured through the nasogastric tube or via Foley catheter measuring intravesical pressure
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Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
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The Montreal group described using the omphalocele sac as a silo by sequentially ligating it over several days to allow abdominal wall stretching
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Sequential sac ligation requires a thick enough sac and umbilical cord insertion at the top rather than the side
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Using Duoderm to gradually reduce omphaloceles appears to achieve reduction more quickly than sac ligation
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Indications for escharotic therapy include prematurity, severe cardiac disease, pulmonary hypoplasia, multiple anomalies, chromosomal abnormalities, or giant omphaloceles where reduction is not feasible
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Mushroom-shaped omphaloceles have a small abdominal wall defect but large external contents, making reduction impossible
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Sigy Ein had long experience using silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles
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With escharotic therapy, the omphalocele is painted with the agent, forms an eschar, eventually epithelializes, and is later repaired like a large ventral hernia
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Timing 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
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For mushroom-shaped omphaloceles, the abdominal wall defect can be enlarged surgically as a first step to allow more spontaneous reduction before definitive repair
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Formal adult-style component separation in small children carries risk of devascularization and can leave patients in worse condition if complications occur
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In omphalocele closure, the defect often extends to the costal margin, making the upper portion impossible to close primarily, requiring patch placement
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Surgisis patch fails in approximately 50% of omphalocele repairs, requiring reoperation with non-absorbable mesh like Prolene
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Omphaloceles can be part of pentalogy of Cantrell, commonly associated with diaphragmatic hernia of Morgagni
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Most pentalogy of Cantrell patients have cardiac problems requiring escharotic therapy for the omphalocele
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In pentalogy of Cantrell, the diaphragmatic patch can be placed from above through sternotomy during cardiac surgery, with abdominal wall repair performed later
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Reflux is very common in omphalocele patients, and many don't eat normally, especially with cardiac disease or pulmonary hypoplasia
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Interventional radiologists can place G-tubes under fluoroscopy in giant omphalocele patients by finding a window lateral to the defect
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