Omphalocele occurs when some of the organs that normally are located in a baby’s abdomen are pushed out into the base of the umbilical cord (see illustration). 

An infant with an omphalocele. in which the abdominal organs are pushed out into the base of the umbilical cord.

The abdominal cavity itself may be small and underdeveloped because the organs normally contained in it developed outside of it. The organs in the defect may be covered by a membranous sac, or the sac may rupture before, during or after delivery. 

Omphaloceles vary in size from very small to "giant." Small omphaloceles occur in one out of every 5,000 to 6,000 births. Large or "giant" omphaloceles occur in one out of every 10,000 live births. 

A small omphalocele involves only a small portion of the intestine outside the body. A giant omphalocele is very large and may involve other organs, such as liver, spleen and intestines. Giant omphaloceles may have a more guarded prognosis than small omphaloceles.

Up to 50% of babies with an omphalocele will have an associated anomaly, or birth defect, including: 

• Cardiac defects (most commonly seen; found in 19-32% of cases)
• Neurological defects
• Skeletal defects
• Chromosomal defects
• Urinary tract defects
• Beckwith-Weidemann syndrome

Omphaloceles associated with other anomalies also may have a more guarded prognosis. 

Omphalocele occurs when some of the organs that normally are located in a baby’s abdomen are pushed out into the base of the umbilical cord (see illustration). 

An infant with an omphalocele. in which the abdominal organs are pushed out into the base of the umbilical cord.

The abdominal cavity itself may be small and underdeveloped because the organs normally contained in it developed outside of it. The organs in the defect may be covered by a membranous sac, or the sac may rupture before, during or after delivery. 

Omphaloceles vary in size from very small to "giant." Small omphaloceles occur in one out of every 5,000 to 6,000 births. Large or "giant" omphaloceles occur in one out of every 10,000 live births. 

A small omphalocele involves only a small portion of the intestine outside the body. A giant omphalocele is very large and may involve other organs, such as liver, spleen and intestines. Giant omphaloceles may have a more guarded prognosis than small omphaloceles.

If your baby is diagnosed with omphalocele, you likely will have to undergo some tests to confirm the diagnosis and check for additional abnormalities, and you will be asked to perform special monitoring later in the pregnancy.

Tests and monitoring include:

• Targeted ultrasound: Doctors can confirm the diagnosis of omphalocele by ultrasound.
• Amniocentesis: Omphaloceles can be associated with some chromosomal abnormalities (up to 30%), so your doctor may offer an amniocentesis.
• Fetal echocardiogram: Your doctor may also recommend an ultrasound of your baby's heart (fetal echocardiogram).
• Regular ultrasounds: If your doctor diagnoses omphalocele, your baby will be monitored closely throughout the pregnancy. Your doctor will perform ultrasounds every 2 to 4 weeks to assess how well your baby is growing and to check the amniotic fluid volume and fetal well-being.
• Fetal movement counting: At about 28 weeks of gestation, your doctor will explain how to do daily fetal movement counting.
• Non-stress tests: Your doctor may schedule non-stress tests (a recording of the baby's heart rate while you are sitting and pressing a button each time the baby moves) at around 32 weeks of gestation.
• Biophysical profiles (BPP): Your doctor may also schedule weekly biophysical profiles (BPP). The BPP is an ultrasound that monitors amniotic fluid volume, your baby's breathing movements, and movements of the extremities.

When the baby is born, the defect in the abdominal wall usually is covered with a membranous sac. However, if this sac is injured, the abdominal organs may be exposed. Exposed organs leave your baby at risk for infection, and the exposed organs can lose heat and fluids. Until surgery, doctors will cover the bowels with moist, warm, sterile gauze, and they will place the lower half of your baby's body in a bag to hold moisture and heat in. If the membrane is intact, placing a dry dressing on top of the membrane is usually sufficient.

An infant with a repaired omphalocele.

If your baby has an omphalocele, they will need surgery. During surgery, the doctor will put the organs back inside your baby's abdominal cavity. If possible, all of the abdominal organs will be put back into the abdominal cavity during this first surgery. This is referred to as a "primary closure." If all of the organs do not fit, immediate surgery may not be possible. The plan then would be to allow the sac to be the protective barrier until the skin grows over it. Surgery would be postponed for 6 months to 1 year, and it would typically be done in stages until final closure. 

Sometimes, as the bowel is moved into the abdomen, babies with this condition require help breathing. 

After surgery, your baby may not be able to breathe effectively on their own due to the increased pressure on the diaphragm. Doctors will place a special tube (called an endotracheal tube or ETT) at the time of surgery (if it was not needed earlier) to assist with breathing. This tube goes through the vocal cords and into the windpipe. While the ETT is in place, your baby will not be able to make any noise because of the tube’s position in the vocal cords. This tube will stay in until your baby can breathe well on their own. 

Doctors also will pass another tube into your baby's stomach (either through the nose or mouth). This tube will be suctioned to keep the stomach empty so as not to cause more pressure on the diaphragm.

Your baby will not be able to eat for a time after surgery. Some special lines are put in place while your baby is in the operating room for surgery. These lines include: 

• A central line will provide nourishment (vitamins, minerals, protein, calories, fat) for a prolonged period of time.
• An arterial line will be placed in one of the extremities. We can monitor blood pressure and remove blood for lab work through this line.
• Before your baby goes to surgery, doctors will place a peripheral IV line. This IV line may be placed in the hand, arm, scalp, foot or lower leg.

There are some other things you can expect during and after surgery, including:

• Your baby will need frequent blood draws for lab work to monitor oxygenation, electrolytes, blood count as well as other things.
• Your baby may be on a variety of medications that can include antibiotics to fight infection and pain medication to control pain after surgery.
• Your baby may look puffy or swollen after surgery. The legs may be especially swollen. This is called edema. It will go away as your baby gets stronger and their body adjusts to the pressure of the bowels being back in the abdomen.

Because some babies with omphalocele cannot eat for a prolonged period of time, doctors will give special nourishment through the central line. Total parenteral nutrition is an IV solution that contains protein, fats, sugar, vitamins and minerals. This will supply your baby with all of their nutritional needs until they are able to take food by mouth.

One of the hardest parts of recovery for babies with omphalocele is learning to eat and tolerating food. Their bowels require time to adjust to being inside the body. We wait for signs from your baby that the bowels are beginning to work. These signs include: 

• Bowel sounds
• Spontaneous passing of stool
• Decrease in the amount of drainage coming from the tube in your baby's stomach

Your baby will go home when they are eating and tolerating enough food to grow. A typical baby with omphalocele will be in the NICU for approximately 4 to 8 weeks. Time in the NICU will depend upon on the size of the omphalocele and the presence and extent of related anomalies. 

If your baby has a giant omphalocele, it takes more time for the final closure and all the intestines to be fit back into the body. Your baby may be in the hospital for 3 to 6 months. A lot of time will be spent helping your baby learn to eat. 

Speech therapists are available to help encourage your baby take a bottle, and lactation consultants are available to help with breastfeeding.