WILL HAPPEN AT BIRTH?
If everything goes as planned, you will deliver at a tertiary
care center with direct access to a neonatal intensive care unit.
The neonatologists will be present at delivery, so that they
can immediately assess your baby and start treatment, if necessary.
At the same time, the pediatric surgeons will be alerted, so
that surgical correction can be performed as soon as possible.
In most cases, however, you will be able to see (and hold) your
baby after delivery.
Your baby will be "stabilized" in the intensive care
unit. An intravenous line will be placed in an arm or a leg,
so that fluids can be given. Because of the exposed intestines,
your baby is likely to lose a lot of fluid by evaporation, and
is likely to cool off more rapidly as well. Your baby will therefore
be placed under a warmer, and if the omphalocele membrane is
ruptured, the loops of bowel will be carefully wrapped to protect
them from the outside. If you baby shows signs of distress, it
is possible that he will be intubated, so that we can help him
If it is clear that there are no other major problems, your baby
will be ready to undergo surgical repair of the defect. How this
is done will depend on how much intestines and other organs are
exposed, and how big your baby is. In many cases, all the intestines
can safely be placed back in the abdomen (so-called "primary
repair"), and the abdominal wall can be closed. Of course,
this is done in the operating room with your baby under anesthesia.
Often, however, there is so much out that this cannot be safely
replaced all at once. In that case, we try at least to protect
the intestines until they are ready to be put back in the abdomen.
For this, we place a "silo" (a clear plastic or silicone
pouch) over the intestines, so that they are now shielded from
trauma, infection and dehydration. This can be done at the bedside,
in the intensive care unit, or in the operating room.
Once the swelling has gone down and the abdomen has become used
to the presence of more bowel, the silo can be removed and the
abdomen closed over the intestines. This typically takes a few
days to a week.
If the membrane around the omphalocele is intact, it acts as
a silo, and surgical intervention can be delayed somewhat. Small
omphaloceles can be treated as described above. With giant omphaloceles,
where a large portion of the liver is exposed, surgical intervention
may be more difficult. If complete correction cannot rapidly
be achieved, the first goal is to close the skin over the abdominal
organs, so that they can be protected. Repair of the muscle defect
may have to occur later.
Of course, associated anomalies may have to be addressed as well;
as discussed, these anomalies (chromosomal or other) may be the
determining factor for the baby's outcome.
WHAT HAPPENS NEXT?
average, it may take 2 to 3 weeks before the intestinal tract
functions properly again. During that time, your baby will be
fed through the veins only, by "total parenteral nutrition,"
or TPN. He will get all the calories necessary to grow, until
he can be fed by mouth again. Once gut function returns, it will
likely take a while before your baby can tolerate full feeds,
and that nutrition through the veins can be stopped. Your baby
is likely to stay in the hospital for at least 1 month. Sometimes,
this can be much longer, depending on the degree of prematurity
and the associated anomalies.
AND LONG-TERM OUTCOME
The overall outcome of isolated omphalocele 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.
As mentioned, omphaloceles can be associated with other conditions.
This happens in approximately 50% of patients with an omphalocele.
If there are associated anomalies, these may have to be addressed
as well. Trisomy 13 and trisomy 18 are severe chromosomal anomalies
with a generally poor prognosis; cloacal exstrophy is not an
immediately life-threatening condition, but is a complex anomaly
that will require multiple surgical interventions and the input
of many specialists. The prognosis of children with Pentalogy
of Cantrell depends mostly on the degree of heart anomaly and
whether the heart is exposed or not.
Beckwith-Wiedemann syndrome, which may present at birth with
pancreas anomalies (too much insulin secretion, resulting in
a very low blood sugar), is important because of its associated
risk of childhood tumors. While most of these tumors can be treated
effectively today, early detection is important. Therefore, babies
with Beckwith-Wiedemann syndrome need to be screened (usually
by ultrasound) on a regular basis for the first few years of