This is an archived article that was published on sltrib.com in 2008, and information in the article may be outdated. It is provided only for personal research purposes and may not be reprinted.
Liz and Jay Sanders had just learned they were going to have a girl. As the young Sandy couple looked at the ultrasound monitor and saw their daughter's profile, they thought she was making a funny face, that she had a recessed chin.
Then the bad news that would lead to their becoming a medical first in Utah: "There's a problem."
Their daughter had a massive growth in her neck. Doctors didn't know if she would be able to breathe once she was born. But they figured it could take from several minutes to an hour to create an airway - causing either brain damage or death.
Over the next four months, a team of doctors and nurses from three hospitals decided to perform surgery in January while the baby was, essentially, partially delivered.
They proposed delivering just the infant's head and upper chest. To keep her alive, she would stay connected to the placenta to continue getting oxygen while doctors intubated her. Called an ex utero intrapartum treatment, or EXIT, it's been done elsewhere in the United States but not here.
As Liz Sanders was being wheeled to the operating room at University Hospital where 20 people would participate in the surgery, the 25-year-old - who had initially wanted a drug-free birth attended by a midwife - thought about the risks.
Since doctors would be "faking out" her uterus so it wouldn't contract, the surgery was riskier than a typical cesarean section. If Sanders was bleeding too heavily, she could awake without a uterus - devastating for the couple who each were one of 10 children and wanted a large family.
Or, if her life was in jeopardy, doctors told Sanders they would let her daughter die.
Too many birth defects
Liz and Jay Sanders could have traveled to San Francisco for the operation, since the University of California, San Francisco invented EXIT in the early 1990s.
But the Utah medical community is trying to expand what it can do to address birth defects in the womb - in addition to treating them after birth.
The reasons: a handful of doctors who have trained at fetal centers like San Francisco and Children's Hospital of Philadelphia now work in Utah.
In addition, doctors say birth defects are on the rise. Utah has the country's highest rate of cleft lips and cleft palates. Abdominal wall defects, called gastroschisis, are also increasing.
In response, University Hospital - which delivers the lion's share of babies with birth defects discovered while in the womb - recently created a Fetal-Neonatal Therapy Program.
The team includes ob-gyns who specialize in complicated pregnancies, radiologists, and pediatricians from Primary Children's Medical Center who specialize in cardiology, surgery and caring for critically ill infants.
The goal of the team is to recognize birth defects during the pregnancy, plan the births and arrange subsequent surgeries to address the defects.
"We're just seeing more and more people referred in with very complicated birth defects," said Janice Byrne, director of the program, which involves doctors from competing hospitals: the U. and Intermountain Healthcare. "We're trying to improve the care to families."
Sanders' EXIT surgery earlier this year, followed by another one the same week, would be team's most complicated test.
It took weeks of researching, planning and practicing.
The trick of the surgery is keeping the uterus relaxed so that it doesn't contract and cut off oxygen to the baby. That's why Sanders -in her 38th week of pregnancy - was put under general anesthesia, which is rare for typical C-sections. And she was even more deeply asleep than other types of surgery require.
Doing so increased the risk she could hemorrhage. To cut down on bleeding, Byrne cut Sanders' uterus open with a tool that staples as it cuts. To stop the uterus from decompressing and to keep the baby warm, Byrne kept as much of the baby inside the womb as possible, pulling her out only to her shoulders.
Then it was pediatric surgeon Eric Scaife's job to ensure the baby could breathe. Under normal circumstances, he would have no more than six minutes to secure an airway. "You hope you can intubate, and the child either makes it or doesn't," he said, recalling how it works without EXIT.
For the Sanders' baby, he technically had hours while the she was on "placental bypass."
"It's as though the cord is working like a heart-lung machine for someone who's having their heart operated on," he said.
While an MRI earlier in the pregnancy showed the mass was about half the size of the baby's head and appeared to invade the esophagus and trachea, it turned out that her airway wasn't affected. She still had to be intubated because, like her mom, she had been deeply anesthetized. Doctors then cut the umbilical cord and delivered the baby girl who spent two weeks in the hospital.
The same week, the team performed another EXIT surgery on a baby who had more complicated problems, including a mass that was protruding from his mouth and a severe heart defect. The baby died shortly after, however, because of other birth defects.
"That was the only chance the infant had, so yeah I think it was worth it," Byrne said. "If we knew how much more severe the heart defect was, there may have been different decisions made"
Despite two EXITs in one week, the fetal therapy team says the surgery will happen rarely - only where there are neck masses, large thyroid or tongue masses that would obstruct the airway.
Nevertheless, EXIT helped cement the new team's relationship, Scaife said.
"It really clarifies our relationships with maternal-fetal medicine. We get lots of kids with all sorts of congenital anomalies. We're all working together to see how those kids can be managed."
The Sanders named their daughter Scarlett. The mass, called a cystic hygroma tumor, is visible under the 3-month-old's tongue and under her left ear and chin. It feels lumpy.
But the cysts aren't cancerous, and doctors will continue to drain them and then inject the space with a mix of alcohol and antibiotics to stop them from growing back. She may eventually need cosmetic surgery.
Sanders said she didn't mind being a "guinea pig," since she and her daughter are healthy. Scarlett, who looks like her dad, smiles a lot and is developing normally. Gazing down at the child, Sanders cradled her close and said: "She's about as perfect as they come."