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OMPHALOCELE

(CONGENITAL EXOMPHALOS)
Omphalocele

WHAT YOU NEED TO KNOW:

What is an omphalocele? An omphalocele is a birth defect that causes your baby's intestines or other organs to form outside his body. The organs stay in a clear sac called the peritoneum after he is born. The umbilical cord is attached to the top of the sac. An omphalocele forms when the baby's abdominal wall does not close completely during pregnancy. Your baby's omphalocele may be mild or severe. Mild means only some intestines are involved. Severe means some organs in his chest may be involved in addition to abdominal organs. Your baby may also have heart problems or other birth defects.

What increases my baby's risk for an omphalocele? Your baby's risk may be higher if his mother smoked cigarettes or drank alcohol during her pregnancy. His risk is also higher if his mother was overweight or obese before her pregnancy.

What do I need to know about delivery?

  • Healthcare providers can usually see an omphalocele on an ultrasound before the baby is born. The omphalocele will be visible on your baby's abdomen after he is born if it was not found on an ultrasound.

  • You will need to deliver your baby at a hospital that has experience with omphaloceles. Your healthcare provider may recommend a vaginal delivery unless other problems mean you need a C-section.

What will happen after my baby is born?

  • A ventilator may be used to give your baby extra oxygen. A ventilator is a machine that breathes for your baby if he cannot breathe well on his own. The extra oxygen can help reduce his risk for long-term breathing problems.

  • A nasogastric tube may be used to relieve pressure. The tube will be placed into your baby's nose and guided down to his stomach. Suction is used to remove fluid and air from your baby's abdomen.

  • Your baby may need to have an IV placed in a vein. Healthcare providers will use the IV to give your baby liquids and medicine.

  • Your baby will be kept warm. He will be placed in a warmer. The warmer will help prevent him from becoming too cold from his exposed organs.

  • The omphalocele will be protected. Your baby's healthcare providers will cover the omphalocele to keep his organs protected. They may place his lower body in a medical bag. The bag is designed to keep your baby warm and prevent dehydration from moisture loss. If the omphalocele will not be treated immediately, it will stay covered. Skin will grow over the omphalocele over time.

  • Antibacterial medicine may be applied. Healthcare providers may use an antibiotic cream or ointment on the omphalocele to prevent a bacterial infection.

  • Tests will be used to plan treatment. Healthcare providers may use x-ray, ultrasound, or other pictures to check your baby's organs. Blood tests will also be used to check for infection and show how well his organs are working.

How is an omphalocele treated? Treatment depends on the size of the omphalocele, your baby's development at birth, and any other birth defects he has. He may need to have a tissue expander placed into his abdomen if it is too small to hold the organs. The size of the expander is increased over time to give his abdomen time to grow. He may need any of the following to repair the omphalocele:

  • Organ reduction means the omphalocele is small enough for healthcare providers to put the organs back into place. The opening is then closed with stitches.

  • A silo is a device used to help put the organs back into your baby's abdomen. The silo is placed over your baby's omphalocele and tightened over a few weeks to help guide the organs into place. The silo will then be removed, and his abdomen will be closed during surgery. The sac may be used as a silo if it is thick enough.

  • Surgery may be needed to put your baby's organs back into his abdomen. If the omphalocele is small, your baby may have surgery soon after he is born. A large omphalocele may need to be treated over time. This will give your baby's abdomen enough time to grow and stretch so the organs will fit inside. It may take 6 to 12 months for your baby's abdomen to grow large enough. He may need immediate surgery if the sac ruptures.

What can I do to manage my baby's omphalocele? You may be able to care for your baby at home while you are waiting for the omphalocele to be treated. Healthcare providers will show you how to handle, dress, and bathe your baby. They will show you how to change your baby's diapers. They will also show you how to change bandages and apply medicines to the skin covering the omphalocele.

What are the risks of an omphalocele?

  • Your baby's intestines may become infected or develop necrosis (tissue death). An organ that is inside the sac may be damaged if the blood vessels that supply blood to the organ are twisted or pinched. Your baby may also be born with heart, spine, or digestion problems. His abdomen may not grow to a normal size.

  • As he grows, your baby may have trouble feeding or breathing. He may not grow as quickly as expected. He may also develop a bowel obstruction (block) or get sick more easily than other children.

What can I do to prevent an omphalocele in a future pregnancy? Take prenatal vitamins as directed. Make sure the vitamins contain 400 micrograms of folic acid. Folic acid helps prevent birth defects such as omphalocele. Do not smoke cigarettes or drink alcohol while you are pregnant. Reach or maintain a healthy weight before you become pregnant. Ask your healthcare provider for other ways to prevent an omphalocele.

When should I seek immediate care?

  • Your baby has a fever.

  • Your baby has green or yellow vomit.

When should I contact my baby's healthcare provider?

  • Your baby has fewer bowel movements than usual or has feeding problems.

  • Your baby has a swollen abdomen or is vomiting.

  • Your baby is crying more than usual, fussy, or difficult to comfort.

  • You have questions or concerns about your baby's condition or care.

CARE AGREEMENT:

You have the right to help plan your baby's care. Learn about your baby's health condition and how it may be treated. Discuss treatment options with your baby's caregivers to decide what care you want for your baby.