What Is Gastroschisis?
Gastroschisis (pronounced gas-tro-'ski-zis) means a hole in the abdominal wall of the fetus. Through this opening, loops of bowel (and sometimes stomach, liver and other organs) protrude outside the abdomen. The term only applies when hole is to the side of the umbilical cord (belly button); when the hole is in the belly button, the condition is called omphalocele. Although both conditions appear the same, each condition has its own features. To learn more about omphalocele, click here. Both forms of abdominal wall defects can be detected by ultrasound from the third month of pregnancy on (14 to 15 weeks). As the pregnancy progresses, diagnosis becomes more accurate: it becomes easier to tell gastroschisis and omphalocele apart.
Prenatal management
Gastroschisis is best treated after the baby is born. We can intervene in other ways, though: with advance knowledge of an abdominal wall defect, it is possible to change the plans for delivery of the baby. One can change the mode, place and time of delivery.
Mode of delivery: If bowel and other organs are outside the abdomen, they could be at an increased risk of damage during normal delivery. Some have therefore advocated Cesarean section ("C-section") for gastroschisis. In fact, the risk of injury is only theoretical, and vaginal delivery does not put the baby at an increased risk of complications. For that reason, many (but not all) physicians now recommend normal delivery, unless there are obstetrical reasons to proceed with a C-section.
Place of delivery: As long as the fetus is inside the womb, the gastroschisis is well protected. After birth, however, the exposed organs have to be protected from trauma, dehydration and infection. The baby can be safely transported to a treatment center, as long as certain precautions are taken. However, if the diagnosis of gastroschisis has been made beforehand, it may be advisable to have the baby be born directly in such a treatment center (i.e., a center with a neonatal intensive care unit and immediate access to a pediatric surgery service).
Time of delivery: With gastroschisis, the exposed bowel is often thickened and appears damaged. Some believe that the longer the bowel remains exposed to amniotic fluid, the more it suffers. Many centers have therefore recommended early delivery (between 35 and 37 weeks of gestation, instead of the normal 40 weeks). Others don't believe that there is not enough evidence for this, and recommend that the baby be born as close to term as possible.
What to expect after birth
The infant with gastroschisis is attended to by neonatologists and pediatric surgeons, who will ensure that the bowel is kept moist, warm and clean. An intravenous line may be inserted, so that fluids and medication can be given. Once the baby is stable and breathing well, repair of the gastroschisis can be performed. How this is done will depend on how much bowel and other organs are exposed, and how big the baby is. In some cases, all the organs can safely be placed back in the abdomen (so-called "primary repair"), and the abdominal wall can be closed. Often, however, it takes several days before a definitive repair can be done. In those cases, a temporary covering of the intestines is placed - the so-called "silo." This sterile bag, placed over the exposed bowel, allows the organs to gradually drop back in the abdomen, over several days, without fear of dehydration or infection. The definitive operation is typically performed under anesthesia in the operating room.
What happens later
The intestines may have suffered somewhat during pregnancy, and they will need some time to recover. On average, it may take 2 to 3 weeks before the intestinal tract functions properly again. During that time, the infant is fed through the veins only, by "total parenteral nutrition," or TPN. Once gut function returns, it will likely take a while before the baby can tolerate full feeds, and that nutrition through the veins can be stopped.
The overall outcome of gastroschisis is excellent: some infants may have minor intestinal problems in the first few months, but will recover from that and lead a completely normal life. Although the belly button may not look perfectly normal, there should be minimal scarring.
In some rare cases, however, there may be some complications. While gastroschisis is usually not associated with other anomalies, there may be intestinal defects in 5 to 10%. These represent in utero "accidents," where a piece of intestine becomes necrotic and disappears. As a result, there may be a missing portion of intestine (intestinal atresia), which will have to be fixed. Often, this is not discovered until a few weeks after birth. An additional operation will then be necessary. Very rarely, a large portion of bowel suffers and dies off. In those rare instances, bowel function may suffer.
Useful Links
Most Fetal Treatment Centers have useful information online. A list of NAFTNet centers, links and their respective website can be found by clicking here.
Other useful sites, including national organizations, patient- and parent support groups, professional societies and governmental agencies, can be found on our resources page. Be aware that, by clicking on these links, you will leave the NAFTNet site. NAFTNet does not endorse the content of these individual web sites.
Below are a few sites that have specific information on gastroschisis:
Images of Gastroschisis
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