Omphalocele
What is it?
An omphalocele is a birth defect in which an infant’s intestine or other abdominal organs are outside of the body because of a hole in the belly button area. It is a condition in which the baby’s intestines, liver or other organs come out of their belly button. They are usually in a clear sac that should not be ruptured.
Photo courtesy of Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.
How is it caused / Epidemiology:
It is caused by a combination of genes or other factors, such as the things the mother comes in contact with in the environment or what the mother eats or drinks, or certain medicines she uses during pregnancy. It occurs in the opening of the central abdominal wall where the umbilical cord meets the abdomen.
● Normal fetus - intestine projects and retracts in the sixth to tenth week.
● Abnormal fetus - the intestine never retracts
● For a “small” amount of projection, it affects 1 in 5,000 infants
● For a larger amount, it is affecting 1 in every 10,000 babies
● It is more likely to affect males than females
Treatment
Omphalocele is treated by surgery, but not always immediately after birth. If the omphalocele is not serve the procedure can be performed bedside by a neonatal surgical team. If the omphalocele is small the baby will undergo a primary repair. This means the herniated organs are placed back into the abdomen and the defect is completely closed in one operation. For babies with giant omphalocele that contain the liver and other organs, a staged repair (involving several steps, also called the Schuster procedure) is needed to gradually return the abdominal contents to the bell. The amount of organs protruding may be so large that there isn’t enough room in your baby’s body to fit them all inside, preventing omphalocele closure. When this happens the “paint and wait” method is used where a sac covering the omphalocele is painted with an antibiotic cream and covered with elastic gauze. The baby’s skin will grow over the sac with time. Also other organs such as the lungs will sometimes be too small since the intestines took too much space. So you will need to wait for the lungs to grow and then fully close the stomach.
Complications / Aftermath
Complications:
· Around 30% of babies with this condition will have a genetic defect too
· They can also have heart defects which most of the time would need surgery
· When this is more severe, they can also have a problem with feeding
Aftermath:
· If the baby has no other side effects or problems, they will be able to make full recovery
· Lung development is a problem that babies with this condition could have to face as they grow up
Long-term outlook for babies born with Omphalocele
Babies who have had small omphaloceles receive follow-up through their pediatrician and the pediatric surgeon. Those without associated defects generally have good long-term outcomes. Babies with giant omphaloceles typically need to be followed more closely by a multidisciplinary team as part of ongoing omphalocele treatment. The pulmonary hypoplasia (small lungs) associated with giant omphaloceles can affect not only breathing, but also heart function, ability to feed, and overall development. This represents a significant long-term health issue.