What Is an Omphalocele?
An omphalocele is a birth defect of the abdominal wall where a baby's intestines, liver, or other organs protrude through the belly button. These organs are contained within a thin, transparent membrane called a sac. This condition is often identified on a prenatal ultrasound and requires specialized medical care immediately after birth to protect the organs and plan for surgical repair.
Understanding the specific features of an omphalocele is key to determining the best course of treatment.
- The protective sac: This membrane covers the protruding organs, shielding them from direct exposure to amniotic fluid in the womb. While this sac offers protection, it is fragile and can rupture, which would require immediate surgery to prevent infection and organ damage.
- Size variation: Omphaloceles range from small, containing only a portion of the intestines, to giant, involving most of the abdominal organs. The size is a critical factor in deciding whether the defect can be repaired in a single surgery or will require a more gradual approach.
- Associated health conditions: Many infants with an omphalocele also have other medical issues, such as heart defects or genetic conditions like Beckwith-Wiedemann syndrome (a disorder that causes overgrowth). Because of this, newborns undergo thorough screening, including heart ultrasounds and genetic tests, to create a comprehensive care plan.
Immediate Care for a Newborn with an Omphalocele
As soon as a baby with an omphalocele is born, a specialized medical team takes immediate action to stabilize the infant. This initial care, often led by a neonatologist in the neonatal intensive care unit (NICU), focuses on protecting the exposed organs, preventing infection, and preparing the baby for treatment.
Key stabilization steps include:
- Protect the omphalocele sac: The sac is immediately covered with a warm, moist, sterile dressing and sealed to prevent it from drying out or getting injured. This covering also reduces heat and fluid loss and acts as a barrier against infection.
- Provide breathing and IV support: Many infants with an omphalocele have small chest cavities and may need help from a breathing machine. An intravenous (IV) line is also started to deliver essential fluids and antibiotics, which are crucial for preventing dehydration and infection.
- Decompress the stomach: A thin, flexible tube is guided through the baby’s nose or mouth into the stomach. This tube gently suctions out air and digestive fluids, which keeps the intestines relaxed, reduces pressure in the abdomen, and prevents vomiting.
- Transfer to a specialized NICU: Once stable, the infant is typically transferred to a children’s hospital with a high-level NICU. Here, a team of experts, including pediatric surgeons, provides continuous monitoring and performs further tests to check for any other health issues.
Primary Repair: A Single-Stage Surgical Solution
For babies with a small omphalocele, surgeons may perform a primary repair. This procedure involves a single operation, usually within the first few days of life, to return the organs to the abdomen and close the opening in the belly wall. This approach is used when the infant is stable and the abdominal cavity is large enough to safely accommodate the organs.
The primary repair procedure is performed in a few key steps:
- Prepare for surgery: The operation is done under general anesthesia, ensuring the baby is asleep and feels no pain. The surgeon begins by carefully removing the protective sac that has been covering the organs.
- Return organs to the abdomen: The surgeon examines the organs for any issues before gently guiding them back into their proper place inside the abdominal cavity. The team ensures there is enough space to avoid putting too much pressure on the baby’s lungs or blood vessels.
- Close the abdominal wall: The final step is to repair the defect. The surgeon stitches the abdominal muscles together and closes the skin over them. If the opening is too large to close without tension, a special patch may be used to cover the hole in the muscle layer before the skin is closed.
Staged Repair: A Gradual Approach for Larger Defects
When an omphalocele is large or the baby's abdomen is too small to hold all the organs at once, a single surgery is too risky. It could cause a dangerous increase in pressure inside the belly, affecting breathing and blood flow. In these cases, surgeons use a gradual, multi-step process known as a staged repair. This approach gives the baby’s body time to grow and adapt as the organs are returned to the abdomen.
This multi-step process involves three main phases:
- Create a temporary silo: Surgeons stitch a sterile, flexible pouch called a silo to the edges of the abdominal wall opening. This silo creates a protective, enclosed container that safely houses the organs outside the body.
- Gradually reduce the silo: Over several days or weeks, the medical team gently tightens or squeezes the silo. This slow, steady pressure encourages the organs to move back into the abdominal cavity as the belly muscles and skin stretch to make room.
- Perform final closure surgery: Once all the organs are back inside the abdomen, the silo is removed in a final operation. The surgeon then closes the muscles and skin of the abdominal wall, completing the repair.
Alternative Management: The 'Paint and Wait' Approach
For infants with giant omphaloceles and severely underdeveloped lungs, even a staged repair may be too dangerous initially. For these most fragile newborns, surgeons may opt for a non-operative strategy called the "paint and wait" approach. This method postpones surgery, allowing the baby to grow bigger and stronger before a major operation is attempted.
This long-term management plan focuses on stability and natural healing:
- "Paint" the sac: The omphalocele sac is left intact and is regularly painted with a topical antimicrobial cream. This prevents infection and helps the sac toughen into a protective covering. The baby's torso is often wrapped with a gentle bandage to secure the dressing and contain the omphalocele.
- "Wait" for natural healing: This phase relies on the body's ability to heal itself. Over many months, the baby's own skin slowly grows inward from the edges to cover the sac. At the same time, the defect itself gradually shrinks, helping to gently guide the organs back toward the abdomen.
- Bridge to a future surgery: This approach avoids the immediate risks of surgery and allows the baby’s abdominal cavity and chest to grow, giving the lungs critical time to mature. After the omphalocele is fully covered with skin, it becomes a stable hernia. A final repair surgery is then planned for when the child is much older, often after their first birthday.
