Abdominal Wall Defects with Dr. Jacob Langer
With Dr. Jack Langer · hosted by Dr. Todd Ponsky · StayCurrentMD
Cued at 13:40 · stops at 14:25 · press play
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Most gastroschisis patients don't have any other associated anomalies, and it's rare to have abnormal chromosomes with gastroschisis
Early papers showed benefit to cesarean section for gastroschisis, but those cesarean sections were usually done early at 36 or 37 weeks, raising the question of whether timing rather than cesarean section itself gave the benefit
Many studies have failed to show an advantage to cesarean section for gastroschisis, and most people nowadays would not do routine cesarean section
There has not been any large randomized trial looking specifically at the issue of early delivery for gastroschisis
Toronto's approach is to deliver gastroschisis patients at around 37 weeks unless they've already gone into spontaneous labor
The mean gestational age of onset of labor is earlier in gastroschisis pregnancies, possibly because of inflammatory mediators produced by the inflamed bowel
Labor induction at 37 weeks is successful most of the time in gastroschisis pregnancies, unlike regular pregnancies
Most evidence, including from the CapsNet database, suggests that delivery in a perinatal center is beneficial for gastroschisis
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
Adrian Bianchi first described bedside closure for gastroschisis
Using forceps at the bedside to push bowel back in can damage the bowel in a squiggling baby
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
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
Anthony Sandler championed the sutureless plastic closure approach after training in Toronto
Some gastroschisis patients become tachypneic with bluish legs after reduction and require intubation by neonatologists an hour or two later
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
Spring-loaded silos (Bentech) create outward pressure forces as you push down, making the defect larger over time
The incidence of intestinal atresia in gastroschisis is between 5 and 10%
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
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
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
There is no good evidence for optimal management of atresia in gastroschisis because it's rare, so approach should be individualized
If bowel looks good without much peel, repair the atresia and reduce at the same sitting; if concerning, drop it back in without repair
Stomas are only brought out when there's necrotic bowel requiring resection and the bowel is not healthy enough to anastomose
The umbilicus is the preferred site for neonatal stomas; prolapse occurs regardless of location
Three weeks is average time to bowel function in gastroschisis, so investigations typically aren't started until 4 weeks
Metoclopramide (Reglan) can be given intravenously as a prokinetic agent in gastroschisis patients with hypomotility
A UK study showed cisapride shortened time to bowel function, but cisapride is no longer available
Toronto is conducting a randomized prospective trial to determine if intravenous metoclopramide can shorten the period of hypomotility in gastroschisis
At 4 weeks without bowel function, start with contrast enema to look for mechanical obstruction, can also do upper GI
If still no resolution at 6 weeks, laparotomy is usually performed; sometimes mechanical obstruction is found, sometimes just adhesions
Going in too early on gastroschisis patients with prolonged ileus is a mistake
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
Omphalocele has a much higher incidence of associated anomalies and chromosomal abnormalities compared to gastroschisis
Karyotype analysis and testing for Beckwith-Wiedemann syndrome are routinely done for omphalocele patients
Small omphaloceles without liver are counterintuitively more likely to be associated with abnormal chromosomes than large omphaloceles
There is no rationale for routine cesarean section, delivery at a perinatal center, or preterm delivery for small omphaloceles
Small omphaloceles are simple to repair surgically
For giant omphaloceles (with significant liver), most recommend cesarean section, though this is not evidence-based
Giant omphaloceles should be delivered at a perinatal center due to need for pediatric surgeon and experienced neonatologists
Pulmonary hypoplasia is associated with giant omphaloceles but is very difficult to diagnose prenatally; some patients require early intubation and respiratory support
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
Lacy also described increase in central venous pressure of more than 4 as a concerning threshold
Intraabdominal pressure can be measured through nasogastric tube or Foley catheter (intravesical pressure); the trend is more important than absolute number
Primary closure is attempted for full-term omphalocele patients without significant cardiac disease, respiratory issues, or pulmonary hypoplasia
The Montreal group described using the omphalocele sac as a silo, sequentially ligating it over days to allow abdominal wall stretching before definitive closure
Sequential sac ligation requires a thick enough sac and umbilical cord coming off the top rather than the side
Duoderm compression appears to achieve reduction more quickly than sac ligation and doesn't require a sac amenable to ligation
Indications for escharotic therapy include prematurity, bad cardiac disease, pulmonary hypoplasia, multiple anomalies, abnormal chromosomes, or omphalocele too giant to reduce
Mushroom-shaped omphaloceles (small abdominal wall defect with large amount of viscera out) are impossible to reduce primarily
Sigy Ein used silver sulfadiazine (Silvadene) for escharotic therapy in omphaloceles for many years; Toronto published long-term follow-up
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
Mushroom-shaped omphaloceles never reduce spontaneously and stay large
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
Phil Gazzetta described the 'flip flop' technique (modification of component separation) for omphalocele closure: lateral incision of anterior sheath only, folding over while attached to posterior sheath, creating single posterior layer
Formal adult-style component separation in small children carries risk of devascularization and worsening the situation
In omphaloceles extending to the costal margin, the upper defect cannot be closed, so inferior defect is closed and a patch placed superiorly
Surgisis patch fails about 50% of the time in omphalocele closure; can be replaced with non-absorbable prolene mesh
Stratus is being used more recently for patches but long-term follow-up is not yet available to compare with Surgisis
Omphaloceles can be part of pentalogy of Cantrell, often with missing pericardium or Morgagni hernia; these tend to be more superiorly placed