Omphalocele
Overview
An omphalocele is a congenital (present at birth) condition in which the abdomen (belly) does not form correctly during a baby’s development. This causes the intestines or other organs to grow in a clear sac outside the body. This sac is typically located at the base of the umbilical cord, which becomes the belly button after birth.
Symptoms of an Omphalocele
The main symptom of this condition is the visible organs that should be inside the belly, appearing in a clear sac outside the abdomen. OB/GYNs can often see this condition on prenatal (pregnancy) ultrasounds before your baby is born. If not detected before birth, doctors will see it at the time of birth.
When to see a doctor
If your OB/GYN notices anything out of the ordinary on your prenatal ultrasounds, they will refer you to a high-risk pregnancy doctor to give you more information about your baby’s health and provide additional testing. You may also see a surgeon during prenatal consultation if the condition is discovered before your baby is born.
Causes of an Omphalocele
An omphalocele can happen due to an abnormality during development in utero. The condition occurs when the intestines or organs are not properly within the abdomen, but instead remain external through the opening in the umbilical cord. It is not fully understood by experts why this happens during fetal development.
Who’s at risk
Babies born with an omphalocele may have other genetic conditions as well, including:
Diaphragmatic hernia (hole in the diaphragm – the muscle between the abdomen and chest)
Down syndrome
Heart problems
Kidney problems
Diagnosis of an Omphalocele
In most cases, your OB/GYN can diagnose an omphalocele during a prenatal (pregnancy) ultrasound, usually around 20 weeks into the baby’s development. If not diagnosed before birth, a physical exam after birth will reveal an omphalocele. If your baby’s doctor diagnoses an omphalocele, your child may need additional tests, such as:
Blood tests. These tests check for other genetic conditions, such as heart defects and Down syndrome, that often occur in babies with an omphalocele.
Ultrasound. This imaging test uses sound waves to create detailed pictures of your child’s organs – including the heart and kidneys – and can help check for other genetic conditions that often occur in babies with an omphalocele.
Treatments for an Omphalocele
If your baby has an omphalocele, they may need surgery depending on the size of the defect. If the omphalocele is small enough (as determined by the surgeon), they will have surgery soon after birth to place their organs back into their abdomen.
During this surgery, your baby will be under general anesthesia (fully asleep). Your baby’s surgeon begins by cutting the sac holding your baby’s organs outside of the body and removing any unhealthy tissue. Then, using an incision in the abdomen, the surgeon places the organs back inside your baby in the usual position. Once complete, the surgeon uses stitches to close the opening in your baby’s belly. A bandage will cover the stitches until the incision heals.
If the omphalocele is very large – called a “giant omphalocele” – it is treated with dressings with silver-based gauze that help thicken the outer, gel-like layer that protects the intestines. This process ultimately results in skin growth over the entire affected area. In this case, the surgeon may wait until 2 to 3 years of age to repair the omphalocele.
Before or after surgery, your baby may also need:
Intravenous (IV) feeding. Your baby may need to receive nutrients through an IV line inserted into a vein before transitioning to breast milk or formula.
Ventilator support. Because returning the intestines into your baby’s abdomen causes pressure on their lungs, your baby may temporarily need a ventilator (breathing machine) to support breathing.
Babies who have a small omphalocele and no other congenital (present at birth) conditions typically do not have any long-term effects after recovering from surgery. Babies who have a larger omphalocele typically require ongoing care from a multidisciplinary team, especially when they also have other congenital conditions. Larger omphaloceles can affect the heart, the ability to feed, and growth and development for the long term. Therefore, it is important that babies with this condition receive ongoing care from an experienced pediatric care team.
After Surgery Care
Your baby’s care team will advise you of any specialized diet or feeding needs to follow for your baby after surgery.
Contact Us
If you suspect that your child may have Omphalocele, make an appointment with an Orlando Health pediatrician today so your child can start on the road to improved health.
