Surgical Case Study: A Two-Step Correction of Infant's Omphalocele

Wrapping and Component Separation Surgery

Physicians:

Patrick Dillon, MD, pediatric surgeon, St. Louis Children’s Hospital (SLCH)

Shawn Larson, MD, pediatric surgery fellow, SLCH

Background: During a routine ultrasound at 19 weeks’ gestation, Sarah and Jason Hanebrink of Brighton, Illinois, learned their baby had an omphalocele. Occurring in approximately one in 5,000 births, an omphalocele is a type of hernia in which the intestine and/or other abdominal organs protrude through a defect in the abdominal wall at the umbilicus. A thin layer of tissue usually covers the defect. According to the National Institutes of Health, approximately 25 to 40 percent of infants with an omphalocele have other birth defects, including sometimes-severe cardiac defects and chromosomal abnormalities.

“Once the omphalocele was discovered, our obstetrician immediately referred us to The Fetal Care Center,” says Sarah Hanebrink. “Physicians there recommended we have a fetal heart echocardiogram and an amniocentesis done. Fortunately, those didn’t indicate any other birth defects.”

Baby Ike was delivered at Barnes-Jewish Hospital on August 27, 2010. He immediately was transported to SLCH’s newborn intensive care unit, where Dr. Dillon examined him.

The surgical challenge: According to Dr. Dillon, the decision about when and how to correct omphaloceles depends on the defect’s size and whether the baby has associated anomalies. “In babies with cardiac defects, treatment of those takes priority,” he says. “For babies in good health with relatively small omphaloceles, their defects can be closed within the first days of life as long as there is enough room in the abdominal cavity to hold the organs.”

Some infants need a silastic sheet of material sewn around their omphaloceles, creating a silo. The intestine and liver rest in the silo, and daily pressure is placed on the organs to reposition them inside the body. Usually within a week or so the abdominal cavity has stretched enough to allow for closure of the defect.

In Ike’s case, a different approach was needed. “Although Ike was in good health, his omphalocele was large, with both his intestine and liver outside of his abdomen. Whenever we tried to compress his organs, he developed apnea, and couldn’t tolerate his feedings because of reflux,” says Dr. Dillon.

To alleviate Ike’s complications, Dr. Dillon chose to apply a topical treatment that promotes epithelialization and then wrapped Ike’s omphalocele with an elastic bandage. “Applying this gentler type of pressure alleviated Ike’s bradycardia, apnea and reflux. Since his omphalocele was covered, I felt there was no urgency to operate on him,” says Dr. Dillon. “A better course of action was to wait several months while the intestine and liver gradually compressed. This approach also would give Ike time to nearly double his birth weight.”

After Ike’s parents received instruction and practice on how to wrap the bandage, Ike went home after spending two months in SLCH’s NICU.

The surgical approach: By January 2011, Ike was ready for surgery. Dr. Dillon chose to perform a component separation procedure, which is described primarily in adult surgical literature for closing abdominal defects such as large ventral hernias.

“The premise for using this technique is that you can gain extra length and mobility by separating the fascia and muscles along certain lines of dissection,” he explains. “By separating some of the muscle layers, there was more room within the abdomen to work, with the result that we didn’t need to use a patch in order to gain closure.”

Dr. Dillon believes that the more conservative approach of gentle compression through wrapping, waiting to perform surgery, and then using the component separation surgical technique helped Ike avoid complications.

“The main worry in closing omphaloceles is that a hernia or defect will develop where you did the repair,” he says. “If we had closed Ike’s omphalocele earlier, we may have had to use a patch or there may have been too much tension, both of which can result in a recurrence.”

The outcome: Ike was doing well at his checkup in late February. “Ike should continue to heal and grow normally without the need for further surgery,” says Dr. Dillon. “And his intestine and liver should be normal and healthy. As he grows, there shouldn’t be any restriction on his activity level. When he gets older and goes to school, he can play on the playground with all the rest of the kids.”

Dr. Dillon believes that access to advanced surgical techniques is not the only advantage for parents of babies with omphaloceles who come to St. Louis Children’s Hospital and the Fetal Care Center. “Basically, we have the whole package that’s needed for these patients, from maternal fetal medicine specialists and obstetricians specializing in high-risk pregnancies to newborn medicine physicians familiar with these problems,” he says. “Fortunately, Ike didn’t have any other birth defects, but many of these babies need the specialized cardiac care and genetic testing available at Children’s Hospital.”

For more information about omphalocele or to speak with a pediatric surgeon, call Children’s Direct at 800.678.HELP (4357) or email at Childrens_Direct@bjc.org.