Understanding omphaloceles
What is an omphalocele?
An omphalocele is a condition where a baby is born with some of their abdominal organs (like intestines or liver) sticking out through the belly button, covered by a thin clear sac. It happens very early in pregnancy when the belly wall doesn’t fully close
- Occurs in about 1 in 4,000–7,000 live births
- The organs are covered by a protective sac, unlike in gastroschisis where no sac is present
- Can be small (just a little intestine) or very large (including liver and other organs)
- Sometimes happens along with other conditions, such as heart problems or chromosome differences
- At some point the baby will need surgery to put the organs back inside and close the belly
Fetal Center: the first step in omphalocele care
Multidisciplinary Evaluation: meet with MFM specialist, pediatric surgeon, neonatologist, and other experts who will care for your baby
Comprehensive Imaging: detailed ultrasounds and other tests when indicated to assess your baby's condition and help plan treatment
Coordinated Delivery Planning: our team works to ensure your baby receives immediate specialized care at birth
How is omphalocele evaluated?
- Regular monitoring with a detailed ultrasound throughout pregnancy is recommended to track changes
- A fetal echocardiogram is important to obtain due to the high incidence of associated heart defects
- A fetal MRI is not routinely obtained, but may be indicated to further evaluate the omphalocele or other structures
- Genetic testing is typically recommended because of an association with chromosomal abnormalities
(Pic: fetal ultrasound with arrow demonstrating a giant omphalocele)
What is the initial treatment?
The first step in management is neonatal resuscitation and stabilization, making sure the baby is able to breathe appropriately and the heart is functioning properly as these kids are often born with underdeveloped lungs (pulmonary hypoplasia) and may have an associated heart defect
Typically, a special IV is placed called a PICC line which will allow for laboratory evaluation, as well as giving fluids and nutrition
The omphalocele will be evaluated by the surgical team. Local wound care will then be applied to minimize infection and to keep the omphalocele membrane clean and moist
(Pic: chest x-ray demonstrating small lungs in a patient with a giant omphalocele)
What are the surgical closure options?
Surgical management depends on the size of the omphalocele and the stability of the child. Small omphaloceles can usually be closed primarily, however, if the child has a major congenital heart defect or other severe anomalies, surgical closure might not be possible until later in life. The covering of the omphalocele allows the surgery to be done in a more delayed fashion, and not as an emergency
The pediatric surgical team will discuss options after evaluation of the omphalocele and discussion with the NICU team regarding how the baby is doing after delivery.
Surgical options include:
- Primary closure
- Staged repair (Silo technique/delayed closure)
- Sclerotherapy (paint and wait)
Primary Closure/Staged Repair
Primary surgical closure: This can be done when the omphalocele is small or moderate size, and the contents can easily be reduced into the abdomen without compromising the baby due to a heart defect or underdeveloped lungs
- Best for small/moderate omphaloceles (only bowel involved)
- Typically happens within the first days of life
- Surgeon returns the organs to the abdominal cavity and closes the abdominal wall in one operation
Staged repair (Silo Technique / Delayed Closure): When the omphalocele is large and the child is stable otherwise, without significant heart or lung issues, the approach can be taken where the omphalocele is gradually closed over time
- Used for large or “giant” omphaloceles that include liver or multiple organs
- A sterile silo (pouch) is placed over the organs, gradually reducing them into the abdomen over days to weeks
- Once reduced, the abdominal wall is closed surgically
Sclerotherapy (Paint and Wait)
Sclerotherapy (Paint and Wait): When the omphalocele is large and/or the baby has other medical issues, like a heart defect or underdeveloped lungs, a topical agent can be applied which will allow new tissue and skin to grow over the omphalocele. The definitive closure is then done months to years later
- Used for large or “giant” omphaloceles that include liver or multiple organs
- The sac is treated with topical agents (e.g., silver sulfadiazine or povidone-iodine) to encourage scar tissue formation
- Over time, skin grows over the sac, creating a ventral hernia
- Definitive abdominal wall reconstruction is done later in infancy or childhood
- It's important to wrap the omphalocele so it's about the size of a soda can, allowing it to stay the same size while the child grows
Watch the video below where Dr. Fenton discusses how to perform wound care on a giant omphalocele.
Sclerotherapy (Paint and Wait)
Over time, skin grows over the sac, creating a ventral hernia. Eventually, the omphalocele "falls" into the abdomen. When this happens, we ask the parent to wrap the abdomen with an ace wrap to gently keep the omphalocele in the abdomen, allowing the abdomen to grow with the child. With time, the abdomen will be able to accommodate all the organs with closure.
In some cases, consultation with plastic surgery may be needed to make sure there is enough skin for closure as the skin overlying the omphalocele often cannot be used. Rarely, skin expanders are placed under the skin to grow more normal skin for closure.
Definitive closure
Definitive abdominal wall reconstruction of a giant omphalocele is done later in infancy or childhood, when the abdomen is able to accommodate the abdominal organs and there is enough "normal" skin for closure.
At Primary Children's Hospital, this is typically done with both a pediatric surgery and pediatric plastic surgeon. It usually is a several hour procedure. Sometimes, a gastrostomy tube is placed at the same time if the child still relies on supplemental tube feedings.
Parents should anticipate a 3 - 5 day hospitalization following surgery, allowing the child to recover bowel function, start nutrition, and for pain control.
Our Pulmonary Hypoplasia Program offers Long-term support
What to expect after discharge from the hospital
Children with a giant omphalocele will need long-term follow-up and support. We have a specialized multidisciplinary care clinic at Primary Children's Hospital for kids born with pulmonary hypoplasia (CDH, large CLM, giant omphalocele, PPH) called the Pulmonary Hypoplasia Program (PHP). Your child will follow-up frequently in this clinic and meet with multiple specialists who are experts in this care and will make sure they are growing and developing normally.
These specialists include:
- Pediatric Cardiologist
- Pediatric Pulmonologist
- Pediatric Surgeon
- Pediatric Neurologist
- Developmental Pediatrician
- Occupational therapist
- Physical therapist
- Speech therapist
- Dietician
(pic: Dr. Fenton with a patient who underwent care, including closure, for a giant omphalocele)
Additional information from omphalocele families
“We Have Seen Many Miracles . . . ” the Healing Journey for a Baby With Omphalocele
“We Have Seen Many Miracles . . . ” the Healing Journey for a Baby With Omphalocele
“We Have Seen Many Miracles . . . ” the Healing Journey for a Baby With Omphalocele
Read a family's journey in the care of their daughter with a giant omphalocele treated through the Grant Scott Bonham Fetal Center at Primary Children's Hospital
Owarrior.org: Additional support online!
“We Have Seen Many Miracles . . . ” the Healing Journey for a Baby With Omphalocele
“We Have Seen Many Miracles . . . ” the Healing Journey for a Baby With Omphalocele
O Warrior helps families affected by an omphalocele diagnosis by being their destination for support, resources, education and awareness
