ADVANCES IN OPEN-FETAL SURGERY MAKE THE DREAM OF TREATING BABIES’ POTENTIALLY LIFE-THREATENING CONDITIONS PREBIRTH A REALITY.
THE PICTURE DISPLAYED ON THE OPERATING-ROOM MONITORS AT TEXAS CHILDREN’S HOSPITAL IN HOUSTON PROVIDES BREATHTAKING DETAIL. BENEATH THE WATCHFUL EYE OF A TINY CAMERA SURVEYING THE INSIDE OF A UTERUS, AN ENTIRE WATERY WORLD SLIDES INTO CRYSTAL-CLEAR VIEW. AS THE CAMERA SCANS, AN OUTSTRETCHED HAND BECOMES VISIBLE, FOLLOWED BY AN ARM, THEN A MOUTH, AND THEN THE OPPOSITE HAND. TINY FLECKS OF WHITE SPIN ACROSS THE MONITOR, SKIN CELLS AWASH IN AMNIOTIC FLUID.
But the attention of Anthony Johnson, DO, is riveted elsewhere -- on a network of blood vessels that merge and separate as they crisscross a single placenta shared by a set of identical twins. Working through a tiny incision, just .15 inches wide, Dr. Johnson uses a fetoscope -- a telescope with a small camera attached to the end -- as a surgical window into the pregnant woman’s uterus.
Looking through that porthole, he strives to decipher which blood vessels are connected and thus endangering the lives of the developing twin boys. “We’re going to take out this one right here,” he says. A green light glows on the monitor, highlighting the spot where he will direct the laser fiber that’s threaded inside the fetoscope. A long beep sounds as he presses the foot pedal beneath the operating-room table. The laser fires, sealing off the errant blood vessel.
DR. JOHNSON AND his surgical colleagues at Texas Children’s are part of a small cadre of U.S. physicians who operate in the evolving -- and, some say, controversial -- field of medicine called maternal-fetal surgery. They work within the limited confines of the uterus itself, maneuvering through tiny incisions to stabilize life-threatening blood-supply imbalances and through larger incisions to remove rapidly growing and life-threatening tumor masses. Some surgeons are even endeavoring, through a federal clinical trial, to repair the less frequently fatal but still potentially debilitating effects of spina bifida, a condition in which the spine fails to fully close during pregnancy.
The field of maternal-fetal surgery is aptly named, reflecting the dual interests at play. For prospective parents, these procedures provide some of the first avenues of treatment for what historically have been devastating diagnoses. But they carry with them inherent risks for the mothers-to-be, including the potential for complications in future pregnancies, particularly when larger incisions are involved. Also, there’s no guarantee of happy news upon the baby’s arrival, which could come prematurely, posing another set of risks.
“Previously, the womb had been off-limits -- like it was a black box,” says Darrell Cass, MD, a fetal surgeon and a codirector of Texas Children’s Fetal Center, which also collaborates with Baylor College of Medicine and St. Luke’s Episcopal Hospital.
“Now we are changing this paradigm,” he says. “We are realizing that these diseases we are treating after the baby is born started well before. And a lot of the damage may already have been done. So it makes sense to try and see if there is some way we can intervene earlier in development.”THE 72-MINUTE SURGERY involving mother-to-be Destinee Brown is the third fetal procedure of the day for Dr. Johnson and his surgical partner, Kenneth Moise Jr., MD. Several hours before, they performed an identical laser treatment to repair the same pregnancy complication -- a rare and life-threatening blood-flow disruption called twin-twin transfusion syndrome -- on another woman, Kim Snyder. In all, the Mo-Jo Team, as the doctors are sometimes dubbed, has completed close to 240 fetal interventions in the last three years, with the first having been at the University of North Carolina. According to data provided last fall, of those, nearly 70 cases involved laser treatment of the transfusion syndrome. And in roughly half of those cases, both babies were born following the laser treatment. In 70 percent of them, one baby survived to delivery.
The first 24 hours after the procedure are the most crucial -- if fetal death is going to occur, it’s most likely to occur then, Dr. Johnson tells me. “I hate that first scan,” he states flatly.
Twenty-four hours pass. The first sonogram shows that Brown’s boys are doing well, squirming and kicking. So are Snyder’s girls. But even with the 24-hour hurdle behind them, the women still face nail-biting weeks to come, during which they’re largely restricted to bed rest between the weekly sonograms. Four weeks after surgery, the news is mixed. Both sets of twins, at or near 28 weeks, are still active. But the slow growth of the smaller of each pair weighs heavily on the mothers-to-be. “This is the biggest emotional roller coaster I have ever been on,” Snyder says. The baby shower for Brown is just a week away. “My double-trouble baby shower,” Brown jokes before becoming silent for a moment. “It’s very tough. I want both of them. They [the clinicians] tell me, ‘Don’t lose hope.’ ”
THE PHYSICIANS involved with maternal-fetal medicine -- typically, pediatric surgeons and maternal-fetal specialists -- are a highly trained and selective group, scarcely numerous enough to fill a small conference room. Many of the surgeons trace their training directly back to the first groundbreaking procedures performed by Michael Harrison, MD, at the University of California, San Francisco (UCSF) more than two decades ago. The fetal-surgery program at the Children’s Hospital of Philadelphia, for example, was launched in 1995 by a surgeon who had trained under Dr. Harrison.
In recent years, though, the field has become more popular. As many as two dozen medical centers now perform at least one type of fetal intervention, says Dr. Johnson, a board member of the North American Fetal Therapy Network (NAFTNet), a voluntary association of medical centers in the United States and Canada.
In 2005 and 2006 combined, at least 760 fetal procedures were performed in the United States and Canada, according to NAFTNet data provided by Dr. Johnson. (And that figure doesn’t include procedures like intrauterine blood transfusions and spina bifida surgeries.) Physicians at Texas Children’s say their center, which opened in 2004, is one of roughly a handful in the United States that perform the full gamut of fetal interventions, including open-fetal surgeries. Those procedures are generally considered the riskiest and most complex because a larger incision is required.
Today, laser treatment for twin-twin transfusion syndrome is one of the more common and studied fetal procedures. According to NAFTNet data, nearly half the 760 fetal-surgery procedures performed in 2005 and 2006 involved laser treatment for the syndrome. Nevertheless, the operation for the life-threatening blood imbalance carries its own set of risks. The condition is a rare one, occurring in only about 1,800 to 2,000 U.S. pregnancies annually. To develop the disease, the twins must be identical and share a single placenta.
For reasons that are unclear, a miscommunication occurs in the twins’ blood supply, resulting in one twin receiving too much blood and the other twin not getting enough, Dr. Johnson says. If the syndrome becomes severe before the 24th week, the risk of death and disability is overwhelming; the chance of delivering two healthy babies is less than 10 percent, he says.
“If we don’t do anything, we know we’re going to lose them,” Dr. Johnson says. “When we talk about this [laser treatment], what we talk about is the probability of getting at least one healthy baby. That’s how bad this disease is.”
In less severe cases, amniotic fluid can be removed from the recipient twin. Still, when the syndrome isn’t immediately severe, the optimal course is less clear, says Timothy Crombleholme, MD, director of the Fetal Care Center of Cincinnati. The risk of inserting a needle to remove amniotic fluid is likely less threatening than that of using a larger-diameter fetoscope, he says. Dr. Crombleholme lays out all the medical options for his patients and then leaves the final decision to them.
It’s very difficult, if not impossible, though, for a pregnant woman to sift through these various complexities and provide informed consent, given the emotional ties she has already formed with her developing child, says George Annas, chair of the Department of Health Law, Bioethics & Human Rights at Boston University School of Public Health.
“These are very difficult decisions -- it’s almost unfair,” he says. “Women will do almost anything for their babies. When you make an offer to them, it’s almost impossible for them to refuse.”
AS THE FIELD of maternal-fetal medicine evolves, more procedures can be performed through the tiniest of incisions, thus reducing the chance of premature delivery, among other risks, says Dr. Harrison, founding director of the fetal center at UCSF. Already, urinary-tract obstructions and benign tumors on the lung can be repaired in that manner, he says.
If the risks related to operating on the uterus were entirely eliminated, a whole new realm of serious medical conditions could be addressed before birth, such as severe hydrocephalus of the brain, says Dr. Cass of Texas Children’s. Research has also indicated that the fetus has unique healing abilities, raising the theoretical possibility that one day facial issues such as cleft palate could be repaired in utero, he explains.
At the moment, though, fetal specialists typically operate on conditions that place the fetus on the cusp of life and death. Within 24 hours of her twins’ transfusion syndrome being diagnosed last summer, Shana Elliott was rushed into a Texas Children’s operating room. “They told us it was a 90 to 100 percent chance we’d lose both of them if we didn’t do anything. It wasn’t even a choice,” she says.
Three months later, Elliott is getting to know her daughters in the neonatal intensive care unit. They’re on the small side -- one pound, nine ounces and four pounds, two ounces -- but are, by all indications, healthy, with emerging pint-size personalities. “Annabelle is pretty laid-back and quiet,” Elliott says, describing the larger of the two. “But I think Andie is going to be our live wire.”