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What is an omphalocele?
During pregnancy, a thin muscle wall protects the baby's abdominal organs. Sometimes intestines and other organs protrude through a weak spot or opening in the wall. This is called a hernia. In some cases, the intestines or other organs protrude into the umbilical cord connecting baby to mother. This type of hernia is called an omphalocele, covered by a transparent, two-layer membrane.
If the size of the opening in the muscle wall is small, a small amount of the intestines may remain outside the baby's abdomen. If the opening is larger, the intestines and a portion of the liver may remain outside the baby's abdomen. This is called a giant omphalocele.
What causes an omphalocele?
Omphalocele occurs in one of 3,200 to one of 10,000 babies delivered. Omphalocele may be associated with chromosomal abnormalities, such as trisomy 13, 18, or 21, and Beckwith-Wiedemann syndrome. About half of babies born with omphalocele will have additional birth defects. When other abnormalities are present, the long-term health of the baby depends on the severity of these defects as well.
How is an omphalocele detected?
Omphalocele is commonly seen during a prenatal ultrasound. It is also detected through a blood test done between 15 and 20 weeks of pregnancy, called "maternal serum alpha-fetoprotein" (AFP). Alpha-fetoprotein (AFP) is a chemical substance made by your baby. During pregnancy, some of AFP passes into the mother's bloodstream and can be safely measured from mother's blood to determine the risk of certain abnormalities. High levels of AFP are associated with a variety of birth defects, including abdominal wall defects. The results are available in three to five days. AFP test has no risk to the baby or pregnancy.
How will this condition affect my baby's health?
If an omphalocele is diagnosed before birth, your doctor will carefully observe you and the baby during pregnancy. This also allows you and your doctors time to discuss a plan for delivery and treatment. Your baby's birth can be scheduled to reduce the chance of complications and to allow for the best treatment options. Most babies with an omphalocele are delivered by Cesarean section. Babies with smaller omphaloceles and no other abnormalities are expected to survive and do well.
How will the omphalocele affect my baby's birth?
All babies with an omphalocele should be delivered at a "tertiary center," a hospital that has the staff and equipment ready to support a baby needing surgery or intensive care. Your baby will need the services of the neonatal intensive care unit (NICU) and a pediatric surgeon.
What will happen after birth?
A small omphalocele may be repaired with a simple surgery. If the omphalocele is large, the omphalocele may be allowed to gradually shrink on its own, covered with a special cream.
Your baby may need more treatments after surgery if the:
- Omphalocele is very large
- Baby also has heart problems
- Baby is extremely premature
- Baby can't tolerate the stress of surgery
Every baby is different, so the scheduling of surgery can vary from days to months. Your baby may also require more surgery at a later time. It is common for a ventilator to be used to help the baby breathe. Some babies with a giant omphalocele have severe breathing problems.
At first, your baby will be fed directly into his or her veins through an intravenous or "IV" tube. Once your baby's stomach and intestines are functioning normally, feedings by mouth with breast milk or special formula will slowly be added. Eventually, feedings by mouth will replace the IV feedings. Mothers who wish to breast feed are encouraged to pump and freeze their breast milk.
The baby will be discharged from the hospital when:
- All feedings are taken by mouth
- The baby is gaining weight
- All other conditions are stabilized
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