This descriptive name is derived from the Greek words "gaster" meaning
"stomach" and "schisis" meaning "a split"; or more precisely,
a "split in the abdominal wall". This congenital malformation occurs with a frequency of
about 1 in 5000 births. It was formerly an unsuspected finding at delivery, although
ultrasonography has made prenatal diagnosis much more the rule than the exception. Before this
century, this condition must have been a daunting finding at the time of delivery, as all or most
of the intestine from the stomach to the distal colon is exposed. Occasionally the urinary bladder
and even the gonads are visible. The liver always remains inside. It is important to note that
there is no covering over any of the organs, and the umbilical cord arises directly from and is
nearly surrounded by normal skin. The defect in the abdominal wall through which the organs
protrude always occurs to the right of the umbilical cord, although the reason for this finding is
poorly understood. The opening may be large or it may tightly constrict the encompassed bowel.
The prenatal finding of a child with gastroschisis is not a cause for
alarm. The survival rate for such infants is between 95% and 98%. Associated problems such as
mental retardation, heart defects, and other serious malformations are very unusual. About 15% of
children with gastroschisis, however, do have an atresia, or a blockage, somewhere in the
intestinal tract which must be repaired at a later time, but this condition is rarely
life-threatening. Occasionally a true gastroschisis is reported as an
omphalocele
, which, although somewhat similar in appearance, is a completely different entity, which
has a much worse prognosis. Although a child with gastroschisis is best treated at a
children’s hospital with a Level III nursery where specialists are found who are familiar with
this and similar problems, extraordinary measures at the time of delivery, such as Caesarian
section, are unnecessary. Delivery may take place at any maternity hospital within reasonable
transport distance from a children’s hospital.
Immediately after birth, an IV is started in the baby and antibiotics are
administered to fight infection. Heat and water are quickly lost from the exposed organs, so they
must be wrapped with warm moistened gauze and covered with clean plastic kitchen wrap to help
retain the heat and moisture. Additional fluids are given through the IV. Surgical repair should
occur soon after the child has been transferred and arrangements can be made. A tube is placed to
empty the stomach, and the bowel is gently emptied of meconium. The bowel is rinsed with warm
saline and carefully placed into the abdominal cavity through the defect, which may need to be
enlarged to allow return of the swollen intestine. The skin is then separated from the abdominal
musculature and each layer individually closed. In some cases, the intestine will not fit into the
abdominal cavity without putting undo pressure on the diaphragm and limiting respiration. In such
cases, the excess intestine is left outside of the body and surrounded with a plastic bag, which
is attached to the skin around the opening. Daily for the next week or so, a little more of the
intestine is squeezed back into the abdomen until it is completely inside. During a brief second
operation, the bag is removed and the musculature and skin closed as usual. Two to six weeks from
final closure are generally required for the intestine to begin normal function and until then,
and for some weeks later, the baby must be fed intravenously through a special IV catheter placed
at the time of the first operation. This catheter can remain indefinitely without the doctors and
nurses having to stick the infant repeatedly with IV needles.
By far the majority of children with gastroschisis go on to lead normal lives and only
rarely develop problems later. Generally, the only reminder that anything was ever wrong is a
small scar next to the navel and perhaps a tiny scar where the IV catheter was located.
This descriptive name is derived from the Greek words "gaster" meaning
"stomach" and "schisis" meaning "a split"; or more precisely,
a "split in the abdominal wall". This congenital malformation occurs with a frequency of
about 1 in 5000 births. It was formerly an unsuspected finding at delivery, although
ultrasonography has made prenatal diagnosis much more the rule than the exception. Before this
century, this condition must have been a daunting finding at the time of delivery, as all or most
of the intestine from the stomach to the distal colon is exposed. Occasionally the urinary bladder
and even the gonads are visible. The liver always remains inside. It is important to note that
there is no covering over any of the organs, and the umbilical cord arises directly from and is
nearly surrounded by normal skin. The defect in the abdominal wall through which the organs
protrude always occurs to the right of the umbilical cord, although the reason for this finding is
poorly understood. The opening may be large or it may tightly constrict the encompassed bowel.