Over the past 10 years or so, three major advances have greatly improved the care of infants with gastroschisis: (1) the defect is now almost always detected antenatally, which allows for appropriate obstetrical and parental preparation; (2) the routine use of the preformed, spring-loaded silo has transformed the care of these infants from an obligatory emergency operation (nearly always in the middle of the night!) to a more relaxed, albeit still urgent, and straightforward bedside procedure; and (3) PICC lines have for the most part supplanted tunneled central venous catheters, which were associated with significantly more infectious and mechanical complications in these newborns who need intermediate- and long-term parenteral nutrition. I am confident that prospective studies will continue to show significant improvement in outcomes.
Infants with gastroschisis have a great deal of “third-space” and insensible fluid losses and therefore require large amounts of intravenous fluids, sometimes up to 80 mL/kg in the first 24 h. Unless tissue perfusion and urine output are monitored closely, there can be serious consequences if adequate replacement for fluid losses is not provided.
Silo placement requires experience, patience, and careful technique to avoid bleeding and bowel injury. If the defect is too small, the fascia (and sometimes skin) should be opened inferiorly in the midline. Fibrous bridges should be carefully lysed with electrocautery or ligated to avoid the bleeding that can result from traumatic disruption when the silo is being placed. At the time of fascial closure, it is important to avoid excessive intra-abdominal pressure and the risk of abdominal compartment syndrome. If it is too tight, it is best to apply a temporary dressing or another silo and delay the repair for a few days. Alternatively, a patch of artificial material can be used at the level of the fascia, but these almost invariably become infected and eventually need to be removed. The best intra-operative sign of excessive pressure is the peak inspiratory pressure needed to maintain adequate minute ventilation: a PIP of 25 or more is too high.
After reduction and fascial repair, return of bowel function can be very prolonged. It has always been assumed that this is due to the obvious injury sustained by the intestine, presumably caused by exposure to amniotic fluid, even though there is no correlation between the apparent degree of injury (induration, foreshortening, exudative peel) and the severity or duration of the bowel dysfunction. An alternative hypothesis posits that the bowel dysmotility is the primary lesion and is therefore the cause of the defect rather than the result (perhaps intestine that is poorly motile in early gestation cannot easily return to the abdominal cavity). Regardless, most infants with gastroschisis eventually respond to slowly advance feeds as tolerated until able to wean from parenteral nutrition, which can take many weeks or, in some cases, months. Infants with gastroschisis who do not tolerate full feeds by 4–6 weeks after fascial closure need to be evaluated for stricture. Contrast enema or upper GI contrast study might reveal a change in caliber of intestine due to an obstruction, but even in the absence of an obvious stricture on an imaging study, one should have a low threshold for empiric laparotomy, lysis of adhesions, and tapering or resection of dilated segments of bowel. Finally, retaining the umbilical stump almost always results in a better cosmetic result than a neo-umbilicus created with a purse-string suture or flap umbilicoplasty. On the other hand, it is never advisable to leave the child with no belly button as this causes anguish for children and their parents and has resulted in lawsuits.