Understanding omphaloceles

---

An omphalocele is a condition where a baby is born with some of their belly 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

• 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 in not obtained routinely, but sometimes when 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)

Primary surgical closure: this can be done when the omphalocele is small or moderate size, but the contents can easily be reduced into the abdomen without compromising the baby due to a heart defect or underdeveloped lungs

• Best for small omphaloceles (only bowel involved)
• Surgeon returns the organs to the abdominal cavity and closes the abdominal wall in one operation, typically within the first days of life

Staged repair (Silo Technique / Delayed Closure): When the omphalocele is large and the patient 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): 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