Brown University

Fetal Medicine @ Brown
WARREN ALPERT MEDICAL SCHOOL OF BROWN UNIVERSITY

The Warren Alpert Medical School of Brown University

Abdominal Wall Defects:
Omphalocele
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WHAT'S GASTROSCHISIS?

WHAT'S OMPHALOCELE?

SUPPORT GROUPS

GLOSSARY

The text below will, hopefully, answer some of the questions you may have regarding this condition. You can scroll down, or go directly to a specific question:

Underlined terms are explained in detail - just click on them. A medical review on this topic was recently published by Dr. Kurkchubasche in Medicine & Health/Rhode Island.

 

WHAT 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 breathe better.
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?


Postoperative scar in an infant born with an omphaloceleOn 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.

 


COMPLICATIONS 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 life.

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